Lipid metabolism — Flashcards

Lipid metabolism US Medical PG Flashcards - Medical Study Cards

Master Lipid metabolism with OnCourse flashcards. These spaced repetition flashcards are designed for medical students preparing for NEET PG, USMLE Step 1, USMLE Step 2, MBBS exams, and other medical licensing examinations.

Lipid metabolism Flashcard Deck - 475 Cards

Flashcard 1: lipoamide

Answer: acyl groups

Flashcard 2: Where does fatty acid oxidation take place?

Answer: mitochondria

Flashcard 3: Where does fatty acid synthesis take place?

Answer: cytoplasm

Flashcard 4: Where does steroid synthesis take place?

Answer: SER

Flashcard 5: Where does cholesterol synthesis take place?

Answer: cytoplasm

Flashcard 6: fatty acid synthesis

Answer: carnitite acyltransferase I

Flashcard 7: ketogenesis

Answer: HMG-CoA synthase

Flashcard 8: cholesterol synthesis

Answer: HMG-CoA reductase

Flashcard 9: lecithin-cholesterol acyltransferase (LCAT)

Answer:

Extra: esterification of cholesterol

Flashcard 10: cholesterol ester transfer protein (CETP)

Answer:

Extra: transfer of cholesterol esters between lipoprotein particles

Flashcard 11: apolipoprotein E

Answer:

Extra: mediates remnant uptake

Flashcard 12: apolipoprotein A-I

Answer:

Extra: activates LCAT

Flashcard 13: apolipoprotein C-II

Answer:

Extra: lipoprotein lipase cofactor

Flashcard 14: apolipoprotein B-48

Answer:

Extra: mediates chylomicron secretion

Flashcard 15: apolipoprotein B-100

Answer:

Extra: binds LDL receptor

Flashcard 16: In general, what is the composition of lipoproteins?

Answer: cholesterol, triglycerides, phospholipids

Flashcard 17: Give the life cycle of a chylomicron.

Answer: created by intestine, delivers dietary TGs to tissues and cholesterol to liver

Flashcard 18: Give the life cycle of VLDL.

Answer: created by liver, delivers hepatic TGs to peripheral tissues

Flashcard 19: Give the life cycle of IDL.

Answer: formed by degradation of VLDL in peripheral tissues, delivers TGs and cholesterol back to liver

Flashcard 20: abetalipoproteinemia

Answer:

Extra: low chylomicron and VLDL synthesisfailure to thrive, steatorrhea, ataxia, night blindnessacanthocytes, lipid accumulation within enterocytesARmicrosomal triglyceride transfer protein

Flashcard 21: Give the life cycle of LDL.

Answer: formed by modification of IDL in peripheral tissues, delivers cholesterol to peripheral tissues

Flashcard 22: hormone-sensitive lipase

Answer:

Extra: degradation of TG stored in adipocytes

Flashcard 23: hepatic TG lipase

Answer:

Extra: degradation of TG remaining in IDL

Flashcard 24: lipoprotein lipase

Answer:

Extra: liberates TGs circulating in chylomicrons and VLDLs

Flashcard 25: Give the life cycle of HDL.

Answer: created by liver and intestine, delivers cholesterol from periphery to liver

Flashcard 26: pancreatic lipase

Answer:

Extra: degradation of dietary TG

Flashcard 27: During starvation, what kind of fuels are used by muscles and liver? Why?

Answer: primarily FFA/ketones, reserving glucose for RBCs

Flashcard 28: What are ketones usually dervied from?

Answer: fatty acids, amino acids

Flashcard 29: How many kcal are produced per gram of fat?

Answer: 9 kcal

Flashcard 30: Why are ketones usually produced?

Answer: oxidation fuel source during starvation

Flashcard 31: Where are ketones usually produced?

Answer: liver

Flashcard 32: hyperchylomicronemia (hyperlipidemia type I)

Answer:

Extra: pancreatitis, hepatosplenomegaly, eruptive/pruritic xanthomaselevated chylomicrons, TGs, and cholesterolARlipoprotein lipase

Flashcard 33: CoA

Answer: acyl groups

Flashcard 34: HMG-CoA reductase

Answer: cholesterol synthesis

Extra: insulin, thyroxineglucagon, cholesterol

Flashcard 35: HMG-CoA synthase

Answer: ketogenesis

Extra:

Flashcard 36: carnitite acyltransferase I

Answer: fatty acid synthesis

Extra: malonyl-CoA

Flashcard 37: What is the effect an increased NADH/NAD+ ratio in the liver?

Answer: inhibit gluconeogenesis, promote fatty acid synthesis leading to hypoglycemia, ketosis, acidosis, and fatty liver changes

Flashcard 38: Give two cell types rich in smooth endoplasmic reticulum (SER).

Answer: hepatocytes and steroid-producing cells of adrenal cortex

Flashcard 39: What functions are performed by the smooth endoplasmic reticulum (SER)?

Answer: steroid synthesis, detoxification of drugs and poisons

Flashcard 40: What are five major components of the plasma membrane?

Answer: phospholipid, sphingolipid, glycolipid, protein, cholesterol

Flashcard 41: What is the function of the peroxisome?

Answer: catabolism of long fatty acids and amino acids

Flashcard 42: Where in the cell does fatty acid synthesis occur? _____

Answer: Cytoplasm

Extra: Watch Fatty Acid Synthesis [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-fatty-acid-synthesis?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 43: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is characterized by the accumulation of _ to _-carbon acyl carnitines in the blood

Answer: 8

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 44: Pregnenolone may be converted to _____ via the enzyme 17α-hydroxylase

Answer: 17-hydroxypregnenolone

Extra: * Teleport piper:17-hydroxylase (converts pregnenolone to 17-hydroxypregnenolone) Watch Congenital Adrenal Hyperplasia (CAH) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-adrenal-disorders-congenital-adrenal-hyperplasia-cah?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrinology/adrenals/acquire?ref=anki

Flashcard 45: Which serum lipoprotein is elevated in type I hyperlipoproteinemia? _____

Answer: Chylomicrons

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 46: Which type of acyl-CoA dehydrogenase is used to breakdown 8-10 carbon fatty acyl-CoA? _____

Answer: Medium (medium chain acyl-CoA dehydrogenase)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 47: All steroid hormones are derivatives of _____

Answer: cholesterol

Extra: https://onlinemeded.org/spa/endocrinology?ref=anki

Flashcard 48: In the cytosol, citrate may be converted to _____ via the enzyme ATP citrate lyase (fatty acid synthesis)

Answer: acetyl-CoA

Flashcard 49: Which lipoprotein mediates reverse cholesterol transport from the periphery to the liver? _____

Answer: HDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 50: Muscle biopsy of a patient with primary carnitine deficiency shows elevated _____

Answer: triglycerides

Extra: https://onlinemeded.org/spa/metabolism?ref=anki Atlas:

Flashcard 51: Malonyl-CoA may be converted to _____ via the enzyme fatty acid synthase

Answer: palmitate

Flashcard 52: Type I lipoproteinemia is due to a(n) _____ or apo-CII deficiency

Answer: LPL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 53: One source of vitamin D (cholecalciferol) is through synthesis from 7-dehydrocholesterol in the presence of _____

Answer: UV light

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Flashcard 54: What is the rate-limiting enzyme of ketogenesis? _____

Answer: HMG-CoA synthase

Flashcard 55: Inside the hepatocyte, cholesterol may be converted to _____ via the enzyme acyl cholesterol acyltransferase (ACAT) for storage

Answer: cholesterol ester

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 56: Which lipid transport enzyme is responsible for the degradation of triglycerides stored in adipocytes? _____

Answer: Hormone-sensitive lipase

Extra: https://onlinemeded.org/spa/surgery-general/pancreas/acquire?ref=anki

Flashcard 57: PCSK9 is a protease produced by the liver that leads to degradation of hepatocyte _____ receptors

Answer: LDL

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Flashcard 58: When light strikes the retina, 11-cis retinal is converted to _____ retinal

Answer: all-trans

Extra: https://onlinemeded.org/spa/pediatrics/ophthalmology/acquire?ref=anki

Flashcard 59: HMG CoA is converted to _____ via the enzyme HMG CoA reductase (cholesterol synthesis)

Answer: mevalonate

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 60: The IDL expresses only apolipoprotein _ and _ on its surface

Answer: B-100

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 61: Acetyl-CoA is converted to _____ via the enzyme HMG-CoA synthase (ketogenesis)

Answer: HMG-CoA

Flashcard 62: Once in the vicinity of adipocytes, apoprotein _____ on the VLDL activates the enzyme lipoprotein lipase (LPL)

Answer: C-II

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 63: Once inside the adipocyte, fatty acids are reconverted into _____

Answer: triglycerides

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 64: Which three lipoproteins is apoprotein C-II found on? _____

Answer: HDL, VLDL, chylomicrons

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 65: Fatty Acid Nomenclature: C18:2 (9,12) is a(n) _-carbon fatty acid with _ double bonds

Answer: 18

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 66: Is scavenger receptor A on macrophages down-regulated by cholesterol? _____

Answer: No

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 67: The _____:sphingomyelin ratio in amniotic fluid is used to screen for lung maturity

Answer: lecithin

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/pulmonology/videos/lung-pathology-special-topics?index=12] https://onlinemeded.org/spa/pulmonology?ref=anki L/S Ratio > 2 = mature

Flashcard 68: Thromboxane A2 is synthesized within platelets by the enzyme COX-_____

Answer: 1

Extra: https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 69: The remnant receptor on the liver binds to _____ of the chylomicron remnant, causing subsequent endocytosis

Answer: ApoE

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) Watch Statins [https://dashboard.sketchy.com/study/medical/courses/medical-pharmacology/units/medical-pharmacology-blood-inflammation/videos/medical-pharmacology-blood-and-inflammation-dyslipidemia-drugs-statins?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 70: Patients with heterozygous familial hypercholesterolemia (1:500) have cholesterol levels of ~ _____ mg/dL

Answer: 300

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 71: _____ deficiency may cause dicarboxylic acidemia/aciduria due to omega oxidation of fatty acids

Answer: Medium chain acyl-CoA dehydrogenase (MCAD)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 72: Type IV familial dyslipidemia is due to hepatic overproduction of _____

Answer: VLDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 73: Once depleted of their triglycerides, chylomicron _____ return to the liver

Answer: remnants

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 74: Arachidonic acids are converted to leukotrienes via the enzyme _____

Answer: 5-lipoxygenase

Extra: https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 75: One non-volatile acid is _____ acid, which is generated from metabolism of phospholipids

Answer: phosphoric

Extra: https://onlinemeded.org/spa/nephrology?ref=anki

Flashcard 76: Free fatty acids (from triglycerides) can be used for energy by the _____ and skeletal muscle

Answer: heart

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/cardiac?ref=anki

Flashcard 77: In preparation for β-oxidation, fatty acids in the cytosol are converted to _____ via the enzyme fatty acyl-CoA synthetase

Answer: fatty acyl-CoA

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 78: The main component of chylomicrons is _____

Answer: triglycerides

Flashcard 79: Palmitate is a(n) _____-carbon fatty acid

Answer: 16

Flashcard 80: Decay accelerating factor (DAF/CD55) and Membrane Inhibitor of Reactive Lysis (MIRL/CD59) are secured to the RBC cell membrane by _____, which serves as an anchoring glycolipid

Answer: GPI

Extra: Watch Intravascular Hemolysis Overiew & Paroxysmal Nocturnal Hemoglobinuria (PNH) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-blood-coagulation/videos/medical-pathophysiology-blood-and-coagulation-normocytic-and-macrocytic-anemias-intravascular-hemolysis-overiew-and-paroxysmal-nocturnal-hemoglobinuria-pnh?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/hematology-and-oncology/videos/normocytic-anemia?index=5] https://onlinemeded.org/spa/immunology?ref=anki Other:

Flashcard 81: What is the mode of inheritance of type III familial dyslipidemia? _____

Answer: Autosomal Recessive

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 82: Sulfatides are converted to galactocerebroside via the enzyme _____

Answer: arylsulfatase A

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 83: The enzyme _____ is deficient in Niemann Pick disease

Answer: sphingomyelinase

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 84: After being absorbed from the GI tract, free cholesterol is packaged into a(n) _____ as cholesterol ester

Answer: chylomicron

Flashcard 85: 17-hydroxypregnenolone may be converted to _____ via the enzyme 17,20-lyase

Answer: dehydroepiandrosterone (DHEA)

Extra: Watch Congenital Adrenal Hyperplasia (CAH) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-adrenal-disorders-congenital-adrenal-hyperplasia-cah?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrinology/adrenals/acquire?ref=anki

Flashcard 86: Lipoprotein lipase is regulated via positive feedback by _____ and apoprotein C-II

Answer: insulin

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 87: What substrate accumulates in the lysosomes of a patient with metachromatic leukodystrophy? _____

Answer: Cerebroside sulfate

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 88: Initially, VLDLs only express apoprotein _____

Answer: B-100

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 89: In the ovaries, _____ cells synthesize pregnenolone from cholesterol via the enzyme cholesterol desmolase

Answer: theca

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/histology/videos/female-reproductive-system?index=5] https://onlinemeded.org/spa/endocrinology?ref=anki

Flashcard 90: The main component of LDL is a core of _____

Answer: cholesterol esters

Flashcard 91: What is the mode of inheritance of Niemann Pick disease? _____

Answer: Autosomal recessive

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 92: Vitamin E deficiency may cause _____ anemia and acanthocytosis (hematologic complications)

Answer: hemolytic

Extra: Watch Vitamin E [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-vitamins-minerals/videos/medical-biochemistry-vitamins-and-minerals-fat-soluble-vitamins-vitamin-e?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/hematology-and-oncology/videos/microcytic-anemia?index=12] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 93: LDL only expresses a single copy of apoprotein _____ on its surface

Answer: B-100

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 94: What two fatty acids are essential in humans? _____

Answer: Linoleic acid (omega-6), linolenic acid (omega-3)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 95: _____ are a physical complex of lipids and proteins (apoproteins)

Answer: Lipoproteins

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 96: The first step of fatty acid synthesis is transport of _____ from the mitochondria to the cytosol through its shuttle

Answer: citrate

Flashcard 97: In the blood, chylomicrons pick up apoprotein _ and _ from circulating HDL

Answer: C-II

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 98: Apoprotein C-II is a cofactor and activator of the enzyme _____

Answer: LPL (lipoprotein lipase)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 99: Which lipid transport enzyme is responsible for the degradation of triglycerides in the small intestine? _____

Answer: Pancreatic lipase

Extra: Watch Lipid Digestion Absorption [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-lipid-digestion-and-absorption?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 100: When membrane phospholipids are in the _____ (cis or trans) configuration, they cause decreased membrane fluidity

Answer: trans

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 101: Diabetic Ketoacidosis can present with _____ serum cholesterol, triglycerides, and free fatty acids

Answer: increased

Extra: * Insulin deficiency -> lipolysis -> lipids -> released into bloodstream Watch Diabetic Ketoacidosis (DKA) & Hyperosmolar Hyperglycemic State (HHS) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-diabetes-diabetic-ketoacidosis-dka-and-hyperosmolar-hyperglycemic-state-hhs?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/crash-course-in-medicine/diabetic-ketoacidosis/acquire?ref=anki

Flashcard 102: Triglycerides are composed of three _ and one _

Answer: fatty acids

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 103: In the lymph, chylomicrons only express apoprotein _____

Answer: B-48

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 104: Type _____ familial dyslipidemia is also known as Dysbetalipoproteinemia

Answer: III

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 105: What cellular organelle is responsible for synthesizing Plasmalogens? _____

Answer: Peroxisome

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org?ref=anki

Flashcard 106: Abetalipoproteinemia is associated with malabsorption due to defective _____ formation, which requires apoB-48

Answer: chylomicron

Extra: https://onlinemeded.org/spa/surgery-general/small-bowel/acquire?ref=anki

Flashcard 107: Apoprotein _____ mediates chylomicron secretion by the intestines

Answer: B-48

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 108: Which adrenal cortex deficiency results in shunting towards the androgen synthesis pathway? _____

Answer: 21β-hydroxylase deficiency

Extra: * causes shunting up and to the right into androgen synthesis pathway Watch Congenital Adrenal Hyperplasia (CAH) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-adrenal-disorders-congenital-adrenal-hyperplasia-cah?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrinology/adrenals/acquire?ref=anki

Flashcard 109: Krabbe disease is due to deficiency of the enzyme _____

Answer: galactocerebrosidase

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 110: Farnesyl PPi is an intermediate of cholesterol synthesis that is important for synthesis of _____ (ETC)

Answer: coenzyme Q

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 111: Cholesterol synthesis begins with _____

Answer: acetyl CoA

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 112: What steroidogenesis enzymes are inhibited by ketoconazole and spironolactone? _____ and cholesterol desmolase

Answer: 17α-hydroxylase

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Flashcard 113: _____-carbon steroids have estrogenic activity

Answer: 18

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 114: Ketogenesis begins with _____, which is provided by β-oxidation of fatty acids

Answer: acetyl-CoA

Flashcard 115: Prostacyclin (PGI2) is synthesized by COX-_____

Answer: 2

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Flashcard 116: What % of plasma cholesterol is esterified by the enzyme LCAT? _____

Answer: 67% (2/3rds)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 117: _____ deficiency is characterized by an inability to break down medium-sized fatty acids

Answer: Medium chain acyl-CoA dehydrogenase (MCAD)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 118: HDL may directly bring cholesterol back to the liver via the _____ receptor (reverse cholesterol transport)

Answer: scavenger receptor B-1

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 119: In adipose tissue, insulin stimulates _ and inhibits _

Answer: fat deposition (increased triglyceride synthesis)

Extra: https://onlinemeded.org/spa/surgery-general/pancreas/acquire?ref=anki

Flashcard 120: Glucocorticoids increase peripheral _____, which provides more glycerol to the liver for gluconeogenesis

Answer: lipolysis

Flashcard 121: Which lipoprotein is apoprotein B-48 found on? _____

Answer: Chylomicrons (and their remnants)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 122: Ceramide trihexoside is converted to glucocerebroside via the enzyme _____

Answer: α-galactosidase A

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 123: What are the three ketone bodies? 1. _____ 2. β-hydroxybutyrate 3. Acetone

Answer: Acetoacetate

Extra: Watch Ketone Bodies [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-ketone-bodies?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] Watch Diabetic Ketoacidosis (DKA) & Hyperosmolar Hyperglycemic State (HHS) [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-ketone-bodies?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrine/diabetes/acquire?ref=anki

Flashcard 124: What do fungal plasma membranes contain that others lack? _____

Answer: Ergosterol

Extra: https://onlinemeded.org?ref=anki

Flashcard 125: Nascent HDL is secreted by the _ and _

Answer: liver

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 126: Cholelithiasis may arise due to _____ phospholipids and/or bile acids

Answer: decreased

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Flashcard 127: Pancreatic _____ are responsible for fat digestion in the small intestine

Answer: lipases

Flashcard 128: Zellweger Syndrome is a(n) autosomal recessive leukodystrophy caused by defective _____

Answer: peroxisome biogenesis

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org/spa/biochemistry?ref=anki

Flashcard 129: Insulin inhibits _ formation in the liver because decreased fatty acid degradation provides less acetyl CoA substrate for _ formation

Answer: ketoacid

Extra: https://onlinemeded.org/spa/endocrine?ref=anki

Flashcard 130: Patients with homozygous familial hypercholesterolemia (very rare) have cholesterol levels of ~ _____ mg/dL

Answer: 700+

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 131: _____ is an arachidonic acid pathway metabolite that causes neutrophil chemotaxis

Answer: LTB4

Extra: https://onlinemeded.org/spa/immunology?ref=anki

Flashcard 132: Are triglycerides polar or nonpolar? _____

Answer: Non-polar

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 133: Which lipoprotein is secreted by intestinal epithelial cells and delivers dietary TGs to peripheral tissue? _____

Answer: Chylomicrons

Extra: Watch Statins [https://dashboard.sketchy.com/study/medical/courses/medical-pharmacology/units/medical-pharmacology-blood-inflammation/videos/medical-pharmacology-blood-and-inflammation-dyslipidemia-drugs-statins?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 134: Pregnenolone may be converted to _____ via the enzyme 17α-hydroxylase

Answer: 17-hydroxypregnenolone

Extra: https://onlinemeded.org?ref=anki

Flashcard 135: In the blood, VLDLs pick up apoprotein _ and _ from circulating HDL

Answer: C-II

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 136: Can a urine test for ketones detect acetoacetate? _____

Answer: Yes

Extra: *DKA confirmed by measuring BHB in the SERUM Watch Ketone Bodies [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-ketone-bodies?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] Watch Diabetic Ketoacidosis (DKA) & Hyperosmolar Hyperglycemic State (HHS) [https://dashboard.sketchy.com/study/medical/courses/medical-biochemistry/units/medical-biochemistry-metabolism/videos/medical-biochemistry-metabolism-lipids-ketone-bodies?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrine/diabetes/acquire?ref=anki

Flashcard 137: Which lipoprotein is formed by modification of IDL and delivers hepatic cholesterol to peripheral tissue? _____

Answer: LDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 138: Carnitine acyltransferase-1 (CPT-1) is regulated via negative feedback by _____

Answer: malonyl CoA

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 139: What substance is the precursor to bile acids, steroids, and vitamin D? _____

Answer: Cholesterol

Extra: https://onlinemeded.org/spa/gastroenterology/gallbladder/acquire?ref=anki

Flashcard 140: What type of bond exists between fatty acids and glycerol (e.g. triglycerides)? _____

Answer: Ester bonds

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 141: What is the rate-limiting enzyme of fatty acid synthesis? _____

Answer: Acetyl-CoA carboxylase

Flashcard 142: Vitamin B5 (pantothenic acid) is an essential component of _____ and fatty acid synthase

Answer: coenzyme A

Extra: https://onlinemeded.org/spa/metabolism/pyruvate-dehydrogenase/acquire?ref=anki

Flashcard 143: The first step in all pathways of adrenocortical hormone synthesis is the conversion of _ to _ by the enzyme cholesterol desmolase

Answer: cholesterol

Flashcard 144: In the plasma, Fe2+ is oxidized to Fe3+ by _____ enzymes (e.g. *hephaestin, ceruloplasmin)

Answer: ferroxidase

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/hematology-and-oncology/videos/microcytic-anemia?index=4] https://onlinemeded.org?ref=anki

Flashcard 145: What is the purpose of conjugating bile acids in the liver? _____

Answer: Increase water solubility

Extra: Watch Lab Evaluation of Liver Injury & Hyperbilirubinemia [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pediatrics-hepatobiliary/videos/medical-pathophysiology-hepatobiliary-gallbladder-and-biliary-tract-disease-lab-evaluation-of-liver-injury-and-hyperbilirubinemia?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/physiology?index=8] https://onlinemeded.org/spa/gastroenterology/gallbladder/acquire?ref=anki

Flashcard 146: Which lipoprotein is secreted by the liver and delivers hepatic TGs to peripheral tissue? _____

Answer: VLDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 147: Glucocorticoids have a(n) _____ effect on peripheral fat tissue

Answer: catabolic

Extra: Sketchy Pharmacology: Thyroid, Pituitary, & Adrenal Watch Glucocorticoids [https://dashboard.sketchy.com/study/medical/courses/medical-pharmacology/units/medical-pharmacology-gi-endocrine/videos/medical-pharmacology-gi-and-endocrine-thyroid-parathyroid-and-adrenal-glucocorticoids?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/immunology?ref=anki

Flashcard 148: _____ deliver triglycerides from the intestine to peripheral tissues

Answer: Chylomicrons

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 149: 17-hydroxypregnenolone may be converted to _____ via the enzyme 17,20-lyase

Answer: dehydroepiandrosterone (DHEA)

Extra: https://onlinemeded.org/spa/endocrine?ref=anki

Flashcard 150: Fatty acid synthesis requires _____, which is provided by the HMP shunt and malic enzyme

Answer: NADPH

Flashcard 151: Which organ is responsible for the import and export of lipoproteins? _____

Answer: Liver

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/cardiac?ref=anki

Flashcard 152: As surfactant production is increased (fetal lung maturation), _ levels increase, while _ levels remain constant

Answer: lecithin

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/pulmonology/videos/lung-pathology-special-topics?index=12] https://onlinemeded.org/spa/pulmonology?ref=anki

Flashcard 153: What substrates accumulate in the lysosomes of a patient with Krabbe disease? _____ and psychosine

Answer: Galactocerebroside

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 154: Where in the cell does β-oxidation occur? _____

Answer: Mitochondria

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 155: _____ HDL particles contain LCAT-generated cholesterol esters

Answer: Mature

Flashcard 156: What type of enzyme cleaves phospholipid ester bonds? _____

Answer: Phospholipases

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 157: A cerebroside attached to an additional sugar and sialic acid is a(n) _____

Answer: ganglioside

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 158: _____ are the main source of cholesterol delivery to peripheral tissues in the body

Answer: LDLs

Flashcard 159: The fungal cell membrane is composed of _____ as opposed to cholesterol

Answer: Ergosterol

Extra: https://onlinemeded.org/spa/microbiology/fungi/acquire?ref=anki

Flashcard 160: Leukotrienes are synthesized from arachidonic acid by _____

Answer: 5-Lipoxygenase

Extra: https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 161: Hypothyroidism may contribute to development of _____ due to dyslipidemia and endothelial dysfunction

Answer: atherosclerosis

Extra: Watch Hypothyroidism Overview & Hashimotos Thyroiditis [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-thyroid-and-parathyroid-hypothyroidism-overview-and-hashimotos-thyroiditis?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrine?ref=anki

Flashcard 162: Progesterone may be converted to _____ via the enzyme 17α-hydroxylase

Answer: 17-hydroxyprogesterone

Flashcard 163: In adipose tissue, triglycerides are broken down into glycerol and fatty acids by the enzyme _____

Answer: hormone sensitive lipase

Extra: https://onlinemeded.org/spa/surgery-general/pancreas/acquire?ref=anki

Flashcard 164: Type IIa familial dyslipidemia is due to either: - absent or defective _____ - defective Apo B-100

Answer: LDL receptors

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 165: Increased levels of cholesterol inside the hepatocyte leads to decreased activity of the enzyme _____

Answer: HMG-CoA reductase

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 166: Niacin (Vitamin B3) _ decreases serum triglycerides by decreasing hepatic _ secretion

Answer: mildly

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) Watch Fibrates, niacin [https://dashboard.sketchy.com/study/medical/courses/medical-pharmacology/units/medical-pharmacology-blood-inflammation/videos/medical-pharmacology-blood-and-inflammation-dyslipidemia-drugs-fibrates-niacin?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 167: The triglycerides inside the chylomicron are broken down into _____ by lipoprotein lipase in the vicinity of the adipocytes (or peripheral tissue)

Answer: fatty acids

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 168: Acetyl-CoA is converted to _____ via the enzyme acetyl-CoA carboxylase (fatty acid synthesis)

Answer: malonyl-CoA

Flashcard 169: Once in the vicinity of adipocytes, apoprotein _____ on the chylomicron activates the enzyme lipoprotein lipase (LPL)

Answer: C-II

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 170: Which lysosomal storage disease may be treated with recombinant enzyme therapy? _____

Answer: Gaucher disease (i.e. recombinant glucocerebrosidase)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 171: A ceramide attached to a sugar (e.g. glucose, galactose) is known as a(n) _____

Answer: cerebroside

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 172: Adipocytes, β cells, and FFAs cause release of _____, which contributes to insulin resistance in obesity

Answer: cytokines ("adipokines")

Extra: Watch Diabetes Mellitus Pathogenesis & Acute Clinical Manifestations [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-diabetes-diabetes-mellitus-pathogenesis-and-acute-clinical-manifestations?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrine/diabetes/acquire?ref=anki

Flashcard 173: The enzyme _____ is deficient in metachromatic leukodystrophy

Answer: arylsulfatase A

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 174: _____ is released from the phospholipid cell membrane by the enzyme phospholipase A2

Answer: Arachidonic acid

Flashcard 175: What lecithin:sphingomyelin ratio is indicative of adequate surfactant production? _____

Answer: > 2

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/pulmonology/videos/lung-pathology-special-topics?index=12] https://onlinemeded.org/spa/pulmonology?ref=anki

Flashcard 176: Pharmacology: Cholestatic Drugs The rate limiting step of bile acid synthesis is done via _____

Answer: cholesterol 7a-hydroxylase

Extra: Watch Gallbladder & Biliary Tract Disease [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pediatrics-hepatobiliary/videos/medical-pathophysiology-hepatobiliary-gallbladder-and-biliary-tract-disease-gallbladder-and-biliary-tract-disease?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/pancreatic-and-biliary-pathology?index=12] https://onlinemeded.org?ref=anki

Flashcard 177: Can glucocorticosteroids still be produced if there is absence of cholesterol desmolase, 21β-hydroxylase, 11β-hydroxylase, or 3β-hydroxysteroid dehydrogenase? _____

Answer: No, a lack of any of these enzymes would block the synthesis of both corticosterone AND cortisol

Flashcard 178: As triglycerides are removed from the VLDLs, the VLDL shrinks, forming _____

Answer: IDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 179: The liver packages triglycerides and cholesterol into _____, which enter the blood

Answer: VLDLs

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 180: What demographic has an increased incidence of Tay-Sachs, Niemann Pick, and some forms of Gaucher disease? _____

Answer: Ashkenazi Jews

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 181: Can mineralocorticoids still be produced if the mineralocorticoid pathway is blocked below the level of DOC (i.e. absence of 11β-hydroxylase or aldosterone synthase)? _____

Answer: Yes, 11-deoxycorticosterone (DOC) and corticosterone both have mineralocorticoid activity

Flashcard 182: Treatment for medium chain acyl-CoA dehydrogenase deficiency includes avoiding _____

Answer: fasting

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 183: Apoprotein A-1 is an activator of the enzyme _____

Answer: LCAT (lecithin cholesterol acyltransferase)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 184: Intracellular accumulation of triglycerides and FFAs contribute to insulin resistance in obesity via _____

Answer: inhibition of insulin signaling

Flashcard 185: Fatty acyl-carnitine is transported from the intermembrane space into the mitochondrial matrix via the _____ transporter

Answer: carnitine

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 186: HDL delivers cholesterol esters directly to the liver via _____ receptors

Answer: scavenger receptor B-1

Extra: Sketchy Pharmacology: Blood & Inflammation (Dyslipidemia drugs) https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 187: Once in the vicinity of the liver, the chylomicron remnant binds the _____ receptor via apoprotein E

Answer: remnant

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 188: Which female sex steroid is responsible for female fat distribution? _____

Answer: Estrogen

Extra: https://onlinemeded.org/spa/endocrinology?ref=anki

Flashcard 189: The first intermediate in cholesterol synthesis is _____ (from HMG CoA)

Answer: mevalonate

Flashcard 190: What substrate accumulates in the lysosomes of a patient with Gaucher disease? _____

Answer: Glucocerebroside

Extra: https://onlinemeded.org/spa/metabolism?ref=anki Atlas:

Flashcard 191: In type 1 diabetics, increased lipolysis leads to an increase in free fatty acids which are converted to _____ in the liver

Answer: ketone bodies

Extra: * lack of insulin -> no inhibition of HSL -> lipolysis Watch Diabetic Ketoacidosis (DKA) & Hyperosmolar Hyperglycemic State (HHS) [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-endocrine/videos/medical-pathophysiology-endocrine-diabetes-diabetic-ketoacidosis-dka-and-hyperosmolar-hyperglycemic-state-hhs?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/surgery-general/pancreas/acquire?ref=anki

Flashcard 192: Pregnenolone may be converted to _____ via the enzyme 3β-hydroxysteroid dehydrogenase

Answer: progesterone

Extra: https://onlinemeded.org/spa/endocrinology/adrenals/acquire?ref=anki

Flashcard 193: What salivary enzyme begins the initial digestion of fats? _____

Answer: Lingual lipase

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/non-visceral-anatomy?index=4] https://onlinemeded.org?ref=anki

Flashcard 194: Cortisol travels in the blood bound to _____

Answer: corticosteroid-binding globulin (CBG)

Extra: https://onlinemeded.org/spa/endocrinology/adrenals/acquire?ref=anki

Flashcard 195: Hormone sensitive lipase is regulated via positive feedback by _____ and cortisol

Answer: epinephrine

Extra: https://onlinemeded.org/spa/surgery-general/pancreas/acquire?ref=anki

Flashcard 196: In naturally occurring fatty acids, double bonds are in the _____ configuration

Answer: cis

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 197: Is glycerol polar or nonpolar? _____

Answer: Polar

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 198: What substance is the primary regulator of membrane fluidity? _____

Answer: Cholesterol

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 199: Type III familial dyslipidemia is due to a defective _____ allele

Answer: Apo E

Extra: https://onlinemeded.org/spa/biochemistry?ref=anki

Flashcard 200: Activation of the coagulation cascade requires the _____ surface of platelets

Answer: phospholipid

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/hematology-and-oncology/videos/coagulation-and-fibrinolysis?index=2] https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 201: Inside intestinal epithelial cells, the products of lipid digestion are _____ to reform the ingested lipid

Answer: re-esterified

Flashcard 202: Testosterone is converted to dihydrotestosterone via the enzyme _____

Answer: 5α-reductase

Extra: Watch Benign Prostatic Hyperplasia (BPH) & Prostate Cancer [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-reproductive-gu/videos/medical-pathophysiology-reproductive-and-gu-male-gu-benign-prostatic-hyperplasia-bph-and-prostate-cancer?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical] https://onlinemeded.org/spa/endocrinology?ref=anki

Flashcard 203: Sphingomyelin is converted to _____ via the enzyme sphingomyelinase

Answer: ceramide

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 204: In Krabbe disease, galactocerebroside and psychosine accumulate in _____ and destroy the myelin sheath

Answer: macrophages

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/lysosomal-storage-diseases?index=6] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 205: Vitamin K promotes γ-carboxylation at _____ residues

Answer: glutamic acid

Extra: Watch Warfarin [https://dashboard.sketchy.com/study/medical/courses/medical-pharmacology/units/medical-pharmacology-blood-inflammation/videos/medical-pharmacology-blood-and-inflammation-anticoagulants-and-thrombolytics-warfarin?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/hematology-and-oncology/videos/coagulation-and-fibrinolysis?index=10] https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 206: Which serum lipid is elevated in type IIa familial dyslipidemia? _____

Answer: Cholesterol

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 207: How much energy (kcal) can be produced from 1g of fat? _____

Answer: 9 kcal

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 208: Type I familial dyslipidemia is characterized by a "_____" layer in supernatant

Answer: creamy

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 209: Which lipoprotein is formed by the degradation of VLDL and delivers TGs and cholesterol to the liver? _____

Answer: IDL

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 210: The oil red O and sudan black stains are useful for staining _____

Answer: lipids

Extra: https://onlinemeded.org/spa/metabolism/glycogen-storage-diseases/acquire?ref=anki Atlas:

Flashcard 211: Biliary tract diseases (e.g. PBC, PSC) are associated with _____ serum cholesterol

Answer: increased

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/pancreatic-and-biliary-pathology?index=7] https://onlinemeded.org?ref=anki

Flashcard 212: Increased levels of cholesterol inside the hepatocyte leads to increased activity of the enzyme _____

Answer: ACAT

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 213: Primary carnitine deficiency is characterized by muscle weakness and hypotonia, which is provoked by prolonged _____ (especially if fasting)

Answer: exercise

Extra: https://onlinemeded.org/spa/biochemistry?ref=anki

Flashcard 214: Enzyme Replacement Therapy for _____ disease requires replacement of IV Glucocerebrosidase

Answer: Gaucher

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/lysosomal-storage-diseases?index=7] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 215: Refsum disease is a(n) autosomal recessive metabolic disorder caused by defective _____-oxidation

Answer: α

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org?ref=anki

Flashcard 216: Which vitamins are fat-soluble? _____

Answer: A, D, E, K

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/physiology?index=16] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 217: Fatty acids, from triglyceride breakdown, are broken down into acetyl CoA via the process of _____

Answer: β-oxidation

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org/spa/endocrine/diabetes/acquire?ref=anki

Flashcard 218: The Peroxisome is unique in that it performs α-oxidation to break down _____

Answer: Phytanic Acid

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org?ref=anki

Flashcard 219: What is the purpose of converting cholesterol into cholesterol ester? _____

Answer: More space-efficient (anhydrous)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 220: _____ CoA is the only fatty acid carbon that may be used to make glucose (via gluconeogenesis)

Answer: Propionyl

Extra: https://onlinemeded.org/spa/metabolism/gluconeogenesis/acquire?ref=anki

Flashcard 221: What classes of steroid hormones are decreased in 21β-hydroxylase deficiency? _____

Answer: Glucocorticoids and mineralocorticoids

Flashcard 222: Fat-soluble deficiencies may occur with syndromes that cause _____ (e.g. cystic fibrosis and sprue)

Answer: steatorrhea

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/physiology?index=16] https://onlinemeded.org/spa/gastroenterology/malabsorption/acquire?ref=anki

Flashcard 223: Cholesterol ester inside of mature HDL may be transferred to VLDL, IDL, or LDL via the enzyme _____

Answer: cholesterol ester transport protein (CETP)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 224: What protein is responsible for the rate limiting step in steroidogenesis? _____

Answer: StAR (Steroidogenic acute regulatory protein)

Extra: https://onlinemeded.org/spa/endocrine?ref=anki

Flashcard 225: Type _____ hyperlipoproteinemia is also known as familial hyperchylomicronemia syndrome

Answer: I

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 226: In the liver, bile acids are conjugated to the amino acids _____ or taurine to form bile salts

Answer: glycine

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/physiology?index=8] https://onlinemeded.org?ref=anki

Flashcard 227: What is the mode of inheritance of type IIa familial dyslipidemia? _____

Answer: Autosomal dominant

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 228: The enzyme _____ is deficient in Tay-Sachs disease

Answer: hexosaminidase A

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 229: Fatty acyl-CoA is broken down into molecules of _____ via the enzyme fatty acyl-CoA dehydrogenase (β-oxidation)

Answer: acetyl CoA

Extra: Watch associated Bootcamp video [https://app.bootcamp.com/med-school/biochemistry/videos/cell-biology?index=5] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 230: What classes of steroid hormones are decreased in 17α-hydroxylase deficiency? _____

Answer: Glucocorticoids and androgens

Flashcard 231: What substrate accumulates in the lysosomes of a patient with Niemann Pick disease? _____

Answer: Sphingomyelin

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 232: What enzyme activates vitamin K? _____

Answer: Epoxide reductase

Extra: https://onlinemeded.org/spa/heme-onc/hemostasis/acquire?ref=anki

Flashcard 233: Which androgen is the most potent? _____

Answer: DHT > testosterone > androstenedione

Flashcard 234: Dehydroepiandrosterone (DHEA) may be converted to _____ via the enzyme 3β-hydroxysteroid dehydrogenase

Answer: androstenedione

Flashcard 235: What is the mode of inheritance of type I hyperlipoproteinemia? _____

Answer: Autosomal recessive

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 236: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency may present in infancy or early childhood with _____, hyperammonemia, lethargy, seizures, coma, and liver dysfunction

Answer: vomiting

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 237: What is the rate-limiting enzyme of fatty acid β-oxidation? _____

Answer: Carnitine palmitoyl transferase-1 (CPT-1)

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 238: One external source of vitamin D (cholecalciferol) in the body is through _____

Answer: ingestion/diet

Extra: * other external source is UV light Watch Osteomalacia & Rickets [https://dashboard.sketchy.com/study/medical/courses/medical-pathophysiology/units/medical-pathophysiology-musculoskeletal-derm/videos/medical-pathophysiology-musculoskeletal-and-derm-bone-osteomalacia-and-rickets?utm_source=anki&utm_medium=partnership&utm_campaign=february_update&utm_content=medical]Watch associated Bootcamp video [https://app.bootcamp.com/med-school/gastroenterology/videos/physiology?index=16] https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 239: Which enzyme of the fatty acid synthesis pathway utilizes NADPH? _____

Answer: Fatty acid synthase

Flashcard 240: Galactocerebroside is converted to _____ via the enzyme galactocerebrosidase

Answer: ceramide

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 241: The acetyl CoA generated through β-oxidation is used for _____ (liver) or the TCA cycle

Answer: ketogenesis

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 242: What is the rate-limiting enzyme in the biosynthesis of adrenocortical hormones? _____

Answer: Cholesterol desmolase

Flashcard 243: Are fatty acids polar or nonpolar? _____

Answer: Non-Polar

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 244: What is the main component of VLDLs? _____

Answer: Triglycerides

Extra: https://onlinemeded.org/spa/metabolism?ref=anki

Flashcard 245: The enzyme _____ is deficient in Gaucher disease

Answer: glucocerebrosidase (β-glucosidase)

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Flashcard 246: _____ causes the accumulation of very-long-chain fatty acids in the adrenals, testes, and white matter, presenting as cognitive, visual, hearing, and motor dysfunction

Answer: Adrenoleukodystrophy

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Flashcard 247: What kind of laboratory measurement is used to measure LDL levels (e.g. to start statin therapy)? _____

Answer: Fasting lipid profile

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Flashcard 248: Which lysosomal storage disease is caused by deficiency of hexosaminidase A? _____

Answer: Tay-Sachs disease

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Flashcard 249: The enzyme _____ is deficient in Krabbe disease

Answer: galactocerebrosidase

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Flashcard 250: How do you calculate LDL? LDL (mg/dL) = _____

Answer: total cholesterol – HDL – (triglycerides/5)

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Flashcard 251: Anorexia = _____cholesterolemia

Answer: Hyper

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Flashcard 252: _ Cholesterol or _ bile salts = ↑ risk of gallstones

Answer: ↑

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Flashcard 253: Characteristics of _____: Hepatosplenomegaly Pancytopenia Bone pain/fractures Failure to thrive and delayed puberty

Answer: Gaucher disease

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Flashcard 254: What is the normal range of Cholesterol, serum (Recommendation)?

Answer: From 0 to 200 mg/dL

Flashcard 255: What is the normal range of Triglycerides, serum?

Answer: From 35 to 160 mg/dL

Flashcard 256: An 18 month old boy present with neurologic regression. -He developed a gradual regression of developmental milestones such as inability to sit with support. -Poor head control, loss of social smile. -Physical exam: enlarge liver and spleen, hypotonia, lack of deep tendon reflexes in all limbs. -Cherry red spot on fundoscopic exam. The patient most likely has an accumulation of which substrate?

Answer: Sphyngomyelin Due to Niemann pick disease (deficiency of sphyngomyelinase) similar symptoms as Taysach without splenomegaly. splenomegaly is absent in Taysach disease. (accumulation of GM2).

Flashcard 257: 35 y/o man comes with skin lesions on his palms. -Patient has yellowish skin nodules over palmar creases. -It has been increasing in size and number over the past several years. -He has small clusters of yellow papules on his elbows, knees, and buttocks. -Biopsy of skin lesion:accumulation of lipd-laden macrophages. -Lack of ApoE3 & ApoE4 in his circulation lipoproteins. -What is the most likely impaired in this patient?

Answer: Chylomicron remnant uptake by the liver cells. or VLDL remants which means he has high accumulation of the two. due to Familial dysbetalipoproteinemia (type 3). (palmar xanthomas)

Flashcard 258: A 3 y/o girl comes with several months of fatigue and difficulty walking. -Physical examination: Decreased power in all extremities but no ataxia. -Cardiac auscultation: 1/6 systolic murmur & an S3 gallop. -Labs: Glucose 37 mg/dL Creatine kinase 304 U/L (normal XX: 10 - 70) Urinalysis: Protein, glucose, ketones, Leukocyte esterase, nitrites: all Negative -Muscle biopsy: Very low carnitine content. -Which substance has deficient synthesis in this patient's disease?

Answer: Acetoacetate (Ketone bodies) Due to Primary carnitine deficiency (impairs fatty acid transport into mitochondria) Symptoms: Cardiac & skeletal muscle cells injury, and impaired ketone body production by the liver during fasting periods.

Flashcard 259: What are the followings in relation to very long chain fatty acids oxidation? -Mitochondria -Rough endoplasmic reticulum -Proteasomes -Lysosomes -Peroxisomes -Golgi Apparatus

Answer: Mitochondria: Doesn't metabolize very long chain of fatty acids. -Rough endoplasmic reticulum: site of sythesis of proteins -Peroxisomes: site of synthesis of complex fatty acids, very long chain fatty acids. -Lysosomes: organelles to destroy/degrading fatty acids, proteins, & nucleic acids. -Proteasomes: Also use for degradation. -Golgi Apparatus: Send out proteins to the rest of the body by vesicular transport.

Flashcard 260: Cultured fibroblast taken from an infant suffering from hypotonia & seizures. -It shows an impaired ability to oxidize very long chain fatty acids (VLCFA) & Phytanic acid. -The defect most likely localized to:

Answer: Peroxisomes due to oxidizing very long chain fatty acids & phytanic acid. They usually metabolize in the peroxisomes. Symptoms: neurological symptoms (seizures & hypotonia)

Flashcard 261: A 8 y/o patient present with hypoglycemia and hypoketotic hypoglycemia. -What is the most likely enzymes deficient? -Which substance has deficient synthesis in this patient's disease?

Answer: Acyl CoA dehydrogenase deficiency due to (MCAD) Medium chain acyl CoA dehydrogenase. -Ketone bodies (Acetoacetate) this disease is a milder form of Primary Carnitine deficiency.

Flashcard 262: What are the followings in relation to Carnitine deficiency? -Acetoacetate -Arachidonic acid -Glutathione -Homocysteine -Lactate -Palmitate

Answer: Acetoacetate: (Ketone body) will be deficient in carnitine deficiency because fatty acid will not be able to transport into mitochondria to break down fatty acid into ketone bodies. -Arachidonic acid: are synthesize of membrane phospholipid. Won't be affected. -Glutathione: Important antioxidant. Can synthesize from amino acid (glutamate , cysteine, & glycine) not fatty acids. Plays role in DNA synthesis and repair. It won't be affected. -Homocysteine: Synthesize from amino acid (methionine). Won't be affected. Need to synthesize to amino acid cysteine (via B6) or back to methionine (via B12) to prevent homocysteinuria. -Lactate: is produced from pyruvate under anaerobic conditions. Won't be affected by carnitine defiency. -Palmitate: Fatty acid that is synthesize from carbs in the cytoplasm. Won't be affected by carnitine deficiency because it doesn't need to be transported to the mitochondria.

Flashcard 263: A 12 y/o girl is involved in a motor vehicle accident. -Abdominal CT is negative -She's incidentally found to have an abormal intra-abdominal mass. -Biopsy of mass reveals ectopic tissue. -On microscopy, these cells have a highly developed smooth endoplasmic reticulum. -These cells most likely receive stimulation from which of the following agents? a. Parathyroid hormone b. Progesterone c. ACTH d. Aldosterone e. Dopamine

Answer: ACTH because they stimulate steroids hormones, which contain a well-developed smooth Endoplasmin reticulum. (cholesterol & fat) a.) PTH: increased calcium, they do not regulate lipids or cholesterol synthesis. b.) Progesterone: is a steroid hormones. It prepares endometrium for conception & helps maintain new pregnancy. But it will not regulate fat and cholesterol synthesis even tough it contains Smooth ER. d.) They have effect on Na & K regulation not lipids & cholesterol regulation.

Flashcard 264: What are the followings defects in relation to hyperlipoproteinemias -ApoC-2 production -Cholesterol esterification in the blood -Chylomicron remnant uptake by liver cells -LDL particle uptake by hepatocytes -Lipoprotein lipase activity

Answer: ApoC-2 Production: Familial chylomicronemia syndrome. Present with acute pancreatitis & eruptive xanthomas. -Cholesterol esterification in the blood: LCAT (Lecithin-cholesterol acyltransferase). will present with low HDL & high free cholesterol in blood. -Chylomicron remnant uptake by liver cells: Familial dysbetalipoproteinemia. Premature atherosclerosis & palmar xanthomas. Defect in ApoE protein. -LDL particle uptake by hepatocytes: Familial hypercholesterolemia. Premature atherosclerosis with tendon xanthelasma. Defect in LDL receptor. - Lipoprotein lipase activity: Familial chylomicronemia syndrome. High chylomicrons. Present with acute pancreatitis with eruptive xanthomas. Defect in lipoprotein lipase & ApoC-2.

Flashcard 265: Accumulation of the followings present with what disease? -Cerebroside sulfate -Galactocerebroside -Globotriaosylceramide -Glucocerebroside -GM2 ganglioside -Sphyngomyelin

Answer: Cerebroside sulfate: metachromic leukodystrophy (peripheral neuropathy and ataxia) "Meta Leuko" -Galactocerebroside: Krabbe disease -Globotriaosylceramide: Fabry disease -Glucocerebroside: Gaucher disease -GM2 ganglioside: Taysache disease -Sphyngomyelin: Niemann pick disease

Flashcard 266: 72 y/o woman found lying on the floor in her home for the past 2 days. -She felt and unjured her right hip, was unable to get up and call for help. -She did not drink anything during that time. -Physical exam: dehydration, right hip tenderness. -Labs: serum glucose 72 mg/dL & positive urine ketones. -Radiographs: righ femoral neck fracture. -Increased activity of what enzymes is most likely contributing to both the serum & urine lab findings?

Answer: Hormone-sensitive lipase because of starvation for 2 days. lipids start breaking down to fatty acids and gycerol. -Glycerol will turn to glucose (via gluconeogenesis). -Fatty acids will turn to ketone bodies, exit in urine.

Flashcard 267: 3 y/o patient present with cherry red pots and loss or motor skills. -Physical exam: no splenomegaly -What is the most likely enzyme deficiency that causes this disease?

Answer: Hexosaminidase A deficiency due to Tay-Sachs disease.

Flashcard 268: Which classes of steroid hormones (2) are decreased in 17-hydroxylase deficiency?_____

Answer: glucocorticoids and androgens

Flashcard 269: What protein is responsible for the rate limiting step in steroidogenesis?_____

Answer: StAR (Steroidogenic acute regulatory protein)

Flashcard 270: What is the mode of inheritance of Niemann Pick disease?_____

Answer: Autosomal recessive

Flashcard 271: _____ deficiency is characterized by an inability to break down medium-sized fatty acids

Answer: Medium chain acyl-CoA deydrogenase (MCAD)

Flashcard 272: In the _____, the HMP shunt provides a source of NADPH for biosynthesis of fatty acids, cholesterol, and steroids

Answer: liver

Flashcard 273: Vitamin B7 (biotin) is a cofactor for the enzyme _____ (fatty acid synthesis)

Answer: acetyl-CoA carboxylase

Flashcard 274: Which membrane-bound organelle is involved in the catabolism of very-long-chain fatty acids, branched-chain fatty acids, amino acids, and ethanol? _____

Answer: peroxisome

Flashcard 275: _____ deficiency is an inherited defect in the transport of long chain fatty acids into the mitochondria

Answer: Primary carnitine

Flashcard 276: Which enzyme of the fatty acid synthesis pathway utilizes NADPH? _____

Answer: Fatty acid synthase

Flashcard 277: Which lysosomal storage disease is characterized by a cherry red spot on the macula and hepatosplenomegaly? _____

Answer: Niemann Pick disease

Flashcard 278: HMG-CoA may be converted to _____ via the enzyme HMG-CoA lyase (ketogenesis)

Answer: acetoacetate

Flashcard 279: Bile acids, derived from _____, are released into the intestinal lumen

Answer: cholesterol

Flashcard 280: PGI2, PGE1, PGE2, PGF2-alpha, and thromboxane A2 are synthesized from arachidonic acid by _____.

Answer: COX-1 and COX-2

Flashcard 281: What do fungal plasma membranes contain that others lack?_____

Answer: Ergosterol

Flashcard 282: The main component of VLDL is _____ (60%)

Answer: triglycerides

Flashcard 283: Fatty acyl-carnitine is transported from the intermembrane space into the mitochondrial matrix via the _____ transporter

Answer: carnitine

Flashcard 284: Carnitine acyltransferase-1 (CPT-1) is regulated via negative feedback by _____

Answer: malonyl CoA

Flashcard 285: Which lysosomal storage disease is characterized by a cherry red spot on the macula and no hepatosplenomegaly? _____

Answer: Tay-Sachs disease

Flashcard 286: Primary carnitine deficiency is characterized by muscle weakness and hypotonia, which is provoked by prolonged _____ (especially if fasting)

Answer: exercise

Flashcard 287: What salivary enzyme begins the initial digestion of fats? _____

Answer: Lingual lipase

Flashcard 288: Thromboxane A2 (TXA2) is synthesized by the platelet enzyme _____

Answer: cyclooxygenase-1 (COX-1) (specific)

Flashcard 289: What substrate accumulates in the lysosomes of a patient with Tay-Sachs disease? _____

Answer: GM2 ganglioside

Flashcard 290: What substrate accumulates in the lysosomes of a patient with Fabry disease? _____

Answer: Ceramide trihexoside (globotriaosylceramide)

Flashcard 291: Thromboxane A2 is synthesized within platelets by the enzyme COX-_____

Answer: 1

Flashcard 292: Which apolipoproteins (4) do chylomicrons express on their surface? _____

Answer: Apolipoproteins A-I, B-48, C-II, and E

Flashcard 293: The first step of fatty acid synthesis is transport of _____ from the mitochondria to the cytosol through its shuttle

Answer: citrate

Flashcard 294: What is the precursor molecule to prostanoids and leukotrienes?_____

Answer: Arachidonic acid

Flashcard 295: Which three lipoproteins is apoprotein B-100 found on? _____

Answer: VLDL, IDL, LDL

Flashcard 296: In the liver, acetoacetate may be converted to _____, which then travels to extrahepatic tissues (ketone body metabolism)

Answer: -hydroxybutyrate

Flashcard 297: _____ -carbon steroids include both the mineralocorticoids and glucocorticoids (progesterone, deoxycorticosterone, aldosterone, and cortisol)

Answer: 21

Flashcard 298: Which lipoprotein is formed by the degradation of VLDL and delivers TGs and cholesterol to the liver? _____

Answer: IDL

Flashcard 299: 7-hydroxylase is regulated via negative feedback by _____

Answer: bile salts

Flashcard 300: What substrate accumulates in the lysosomes of a patient with Niemann Pick disease? _____

Answer: Sphingomyelin

Flashcard 301: What is the effect of glucagon on blood fatty acid and ketoacid concentration?_____

Answer: Increased, due to increased lipolysis

Flashcard 302: What substrate accumulates in the lysosomes of a patient with metachromatic leukodystrophy? _____

Answer: Cerebroside sulfate

Flashcard 303: Glucocorticoids have a _____ effect on peripheral fat tissue (anabolic/catabolic)

Answer: catabolic

Flashcard 304: How much energy (kcal) can be produced from 1g of fat? _____

Answer: 9 kcal

Flashcard 305: Which lipoprotein is apoprotein B-48 found on? _____

Answer: Chylomicrons (and their remnants)

Flashcard 306: Which serum lipid is elevated in type IIa familial dyslipidemia? _____

Answer: Cholesterol

Flashcard 307: GM2 ganglioside is converted to GM3 via the enzyme _____

Answer: hexosaminidase A

Flashcard 308: When membrane phospholipids are in the _____ (cis or trans) configuration, they cause decreased membrane fluidity

Answer: trans

Flashcard 309: Fatty acids, from triglyceride breakdown, are broken down into acetyl CoA via the process of _____

Answer: -oxidation

Flashcard 310: What stain is used to screen for fecal fat? _____

Answer: Sudan stain

Flashcard 311: As triglycerides are removed from the VLDLs, the VLDL shrinks, forming _____

Answer: IDL

Flashcard 312: Which female sex steroid is responsible for female fat distribution?_____

Answer: Estrogen

Flashcard 313: Where in the cell does -oxidation occur?_____

Answer: Mitochondria

Flashcard 314: Decay accelerating factor (DAF/CD55) and Membrane Inhibitor of Reactive Lysis (MIRL/CD59) are secured to the RBC cell membrane by _____, which serves as an anchoring glycolipid

Answer: GPI

Flashcard 315: Arachidonic acids are converted to leukotrienes via the enzyme _____

Answer: 5-lipooxygenase

Flashcard 316: Where does the energy to power gluconeogenesis come from in a starvation state? _____

Answer: -oxidation of fatty acids

Flashcard 317: What form of estrogen is produced in the adipose tissue?_____

Answer: Estrone

Flashcard 318: What substance is the primary regulator of membrane fluidity? _____

Answer: Cholesterol

Flashcard 319: Which enzyme is unique to the zona glomerulosa and thus the mineralocorticoid synthesis pathway?_____

Answer: aldosterone synthase

Flashcard 320: ACTH increases steroid hormone synthesis via stimulation of the enzyme _____, thus increasing the conversion of cholesterol to pregnenolone

Answer: cholesterol desmolase

Flashcard 321: What is the rate-limiting enzyme of fatty acid -oxidation? _____

Answer: Carnitine palmitoyl transferase-1 (CPT-1)

Flashcard 322: What is the mode of inheritance of type I hyperlipoproteinemia?_____

Answer: Autosomal recessive

Flashcard 323: _____ hydrolyzes arachidonic acid from the cell membrane

Answer: Phospholipase A2

Flashcard 324: HDL may directly bring cholesterol back to the liver via the _____ receptor (reverse cholesterol transport)

Answer: scavenger receptor B-1

Flashcard 325: Which lipid transport enzyme is responsible for the degradation of triglycerides stored in adipocytes? _____

Answer: Hormone-sensitive lipase

Flashcard 326: Which leukotriene attracts and activates neutrophils?_____

Answer: Leukotriene B4

Flashcard 327: What cellular organelle is responsible for synthesizing Plasmalogens?_____

Answer: Peroxisome

Flashcard 328: Where in the cell does ketogenesis occur?_____

Answer: Mitochondria

Flashcard 329: A cerebroside attached to an additional sugar and sialic acid is a _____

Answer: ganglioside

Flashcard 330: In addition to progesterone, theca cells also synthesize _____ which diffuses to nearby granulosa cells

Answer: androstenedione

Flashcard 331: Conversion of malate to pyruvate via malic enzyme produces a molecule of _____ (fatty acid synthesis)

Answer: NADPH

Flashcard 332: What is the rate-limiting enzyme in the biosynthesis of adrenocortical hormones?_____

Answer: Cholesterol desmolase

Flashcard 333: Vitamin K promotes _____ of factors II, VII, IX, X and protein C/S

Answer: -carboxylation

Flashcard 334: _____ CoA is the only fatty acid carbon that may be used to make glucose (via gluconeogenesis)

Answer: Propionyl

Flashcard 335: Which lipoprotein is formed by modification of IDL and delivers hepatic cholesterol to peripheral tissue? _____

Answer: LDL

Flashcard 336: In naturally occuring fatty acids, double bonds are in the _____ configuration (cis or trans)

Answer: cis

Flashcard 337: Vitamin K is necessary for _____ of factors II, VII, IX, X, and proteins C and S

Answer: gamma carboxylation

Flashcard 338: Testosterone is converted to dihydrotestosterone via the enzyme _____

Answer: 5-reductase

Flashcard 339: Palmitate is a _____-carbon (#) fatty acid

Answer: 16

Flashcard 340: Which serum lipids (2) are elevated in type I hyperlipoproteinemia? _____

Answer: Triglycerides and cholesterol

Flashcard 341: Is cholesterol polar or nonpolar?_____

Answer: Non-Polar

Flashcard 342: The Peroxisome is unique in that it performs -oxidation to break down _____

Answer: Phytanic Acid

Flashcard 343: What is the rate-limiting enzyme of fatty acid synthesis? _____

Answer: Acetyl-CoA carboxylase

Flashcard 344: One source of vitamin D (cholecalciferol) is through synthesis from 7-dehydrocholesterol in the presence of _____

Answer: UV light

Flashcard 345: Are triglycerides polar or nonpolar?_____

Answer: Non-polar

Flashcard 346: Cholesterol from the liver may be repackaged into the interior of _____

Answer: VLDLs

Flashcard 347: The Peroxisome is a cytosolic membrane involved in the -oxidation of _____

Answer: Very-long-chain fatty acids (VLCFA)

Flashcard 348: Which cellular organelle is responsible for the synthesis of cholesterol and bile acids?_____

Answer: Peroxisome

Flashcard 349: A ceramide attached to a sugar (e.g. glucose, galactose) is known as a _____

Answer: cerebroside

Flashcard 350: Ketogenesis begins with _____, which is provided by -oxidation of fatty acids

Answer: acetyl-CoA

Flashcard 351: Muscle biopsy of a patient with primary carnitine deficiency shows elevated _____

Answer: triglycerides

Flashcard 352: LDL only expresses a single copy of apoprotein _____ on its surface

Answer: B-100

Flashcard 353: What type of enzyme cleaves phospholipid ester bonds?_____

Answer: phospholipases

Flashcard 354: Ceramide trihexoside is converted to glucocerebroside via the enzyme _____

Answer: -galactosidase A

Flashcard 355: Is cholesterol ester polar or nonpolar? _____

Answer: Non-polar

Flashcard 356: 1,25-dihydroxycholecalciferol increases _____ absorption of Ca2+ through synthesis of calbindin D-28K

Answer: intestinal

Flashcard 357: Increased levels of cholesterol inside the hepatocyte leads to _____ LDL-receptor gene expression

Answer: decreased

Flashcard 358: Activation of the coagulation cascade requires the _____ surface of platelets

Answer: phospholipid

Flashcard 359: Leukotrienes are synthesized from arachidonic acid by _____.

Answer: 5-Lipoxygenase

Flashcard 360: Insulin inhibits _____ formation in the liver because decreased fatty acid degradation provides less acetyl CoA substrate for ketoacid formation

Answer: ketoacid

Flashcard 361: Rank the forms of estrogen from most to least potent?_____

Answer: 17-estradiol > estrone > estriol

Flashcard 362: Free fatty acids can be converted back to _____ and stored in adipose tissue

Answer: triglycerides

Flashcard 363: What is the rate-limiting enzyme of ketogenesis? _____

Answer: HMG-CoA synthase

Flashcard 364: Which serum lipoprotein is elevated in type IIa familial dyslipidemia? _____

Answer: LDL

Flashcard 365: Where in the cell does fatty acid synthesis occur?_____

Answer: Cytoplasm

Flashcard 366: _____ is associated with absent plasma VLDL and LDL, which require apoB-100

Answer: Abetalipoproteinemia

Flashcard 367: In the first step of testosterone synthesis, cholesterol is converted to pregnenolone via the enzyme _____

Answer: cholesterol desmolase

Flashcard 368: What two fatty acids are essential in humans?_____

Answer: linoleic acid (omega-6), linolenic acid (omega-3)

Flashcard 369: Kwashiorkor may present as liver malfunction with fatty change due to decreased _____ synthesis

Answer: apolipoprotein

Flashcard 370: Following binding of LDL to its LDL receptor, the ligand-receptor complex is endocytosed in _____-coated pits

Answer: clathrin

Flashcard 371: The main component of LDL is a core of _____

Answer: cholesterol esters

Flashcard 372: Dyslipidemia drugs _____ converts free cholesterol into cholesterol esters for transport by HDL

Answer: Lecithin cholesterol acyltransferase (LCAT)

Flashcard 373: The oil red O and sudan black stains are useful for staining _____

Answer: lipids

Flashcard 374: Which steroid synthesis enzyme deficiency results in salt wasting and hyponatremia due to lack of aldosterone? _____

Answer: 21-hydroxylase deficiency

Flashcard 375: Which lipoprotein mediates reverse cholesterol transport from the periphery to the liver? _____

Answer: HDL

Flashcard 376: Pancreatic _____ are responsible for fat digestion in the small intestine

Answer: lipases

Flashcard 377: What is the effect of alcohol on HDL synthesis? _____

Answer: Increased synthesis

Flashcard 378: Placental _____ deficiency is the inability to synthesize estrogens from androgens

Answer: aromatase

Flashcard 379: Dyslipidemia drugs _____ deliver triglycerides directly from the liver to peripheral tissues

Answer: VLDLs

Flashcard 380: What is the rate-limiting enzyme for cholesterol synthesis?_____

Answer: HMG CoA reductase

Flashcard 381: Thromboxane A2 is released upon platelet degranulation and causes platelet _____ and activation

Answer: aggregation

Flashcard 382: Which androgen is associated with prostate growth? _____

Answer: Dihydrotestosterone (DHT)

Flashcard 383: What is the mode of inheritance of type IIa familial dyslipidemia?_____

Answer: Autosomal dominant

Flashcard 384: Abetalipoproteinemia is associated with malabsorption due to defective _____ formation, which requires apoB-48

Answer: chylomicron

Flashcard 385: Can glucocorticosteroids still be produced if there is absence of cholesterol desmolase, 21-hydroxylase, 11-hydroxylase, or 3-hydroxysteroid dehydrogenase? _____

Answer: No, a lack of any of these enzymes would block the synthesis of both corticosterone AND cortisol

Flashcard 386: What % of plasma cholesterol is esterified by the enzyme LCAT? _____

Answer: 67% (2/3rds)

Flashcard 387: Which androgen is the most potent?_____

Answer: DHT > testosterone > androstenedione

Flashcard 388: _____ bring the products of lipid digestion into contact with the intestinal brush border, where the lipid products diffuse across the luminal membrane

Answer: Micelles

Flashcard 389: One function of bile is excretion of _____

Answer: cholesterol

Flashcard 390: What is the purpose of conjugating bile acids in the liver? _____

Answer: Increase water solubility

Flashcard 391: The theca cells and granulosa cells, together, synthesize _____

Answer: estradiol (estrogen)

Flashcard 392: Which serum lipid is elevated in type IV familial dyslipidemia? _____

Answer: Triglycerides

Flashcard 393: Which lipid transport enzyme is responsible for the degradation of triglycerides in the chylomicrons and VLDLs? _____

Answer: Lipoprotein lipase

Flashcard 394: Which part of Lipopolysaccharide is highly toxic and triggers cytokine release?_____

Answer: Lipid A

Flashcard 395: The Fe3+ iron in the plasma is bound to _____ for transport in the blood

Answer: transferrin

Flashcard 396: What type of enzyme cleaves triglyceride ester bonds?_____

Answer: lipases

Flashcard 397: _____ are a physical complex of lipids and proteins (apoproteins)

Answer: Lipoproteins

Flashcard 398: What is the main component of chylomicrons?_____

Answer: Triglycerides

Flashcard 399: Glucocorticoids increase peripheral _____, which provides more glycerol to the liver for gluconeogenesis

Answer: lipolysis

Flashcard 400: What type of bond exists between fatty acids and glycerol (e.g. triglycerides)? _____

Answer: Ester bonds

Flashcard 401: Biliary tract diseases (e.g. PBC, PSC) are associated with _____ serum cholesterol

Answer: increased

Flashcard 402: Initially, VLDLs only express apoprotein _____

Answer: B-100

Flashcard 403: COX-_____ is constitutively expressed

Answer: 1

Flashcard 404: In the lymph, chylomicrons only express apoprotein _____

Answer: B-48

Flashcard 405: Which lipoprotein is a repository for apoproteins C and E? _____

Answer: HDL

Flashcard 406: Which three lipoproteins is apoprotein C-II found on? _____

Answer: HDL, VLDL, chylomicrons

Flashcard 407: Leukotriene _____ and 5HETE are potent chemoattractants for numerous inflammatory cells (particularly neutrophils)

Answer: B4

Flashcard 408: Is scavenger receptor A on macrophages down-regulated by cholesterol?_____

Answer: No

Flashcard 409: Is glycerol polar or nonpolar? _____

Answer: Polar

Flashcard 410: _____ is an arachidonic acid pathway metabolite that causes neutrophil chemotaxis

Answer: LTB4

Flashcard 411: What happens to apoprotein C-II on the VLDL as it forms an IDL? _____

Answer: Recycled back to HDL

Flashcard 412: Which steroid hormone enzyme deficiency presents with increased renin activity?_____

Answer: 21-hydroxylase

Flashcard 413: Bile acid resins (e.g. cholestyramine) interrupt bile acid recycling and promote synthesis of new bile acids, depleting liver _____ stores

Answer: cholesterol

Flashcard 414: The LDL-containing clathrin-coated pits fuse with _____, which break down cholesterol ester into cholesterol

Answer: lysosomes

Flashcard 415: One non-volatile acid is _____ acid, which is generated from metabolism of phospholipids

Answer: phosphoric

Flashcard 416: What happens to apoprotein C-II on the chylomicron as it forms a chylomicron remnant? _____

Answer: Recycled back to HDL

Flashcard 417: Cholesterol synthesis begins with _____

Answer: acetyl CoA

Flashcard 418: HMG CoA reductase requires two molecules of _____

Answer: NADPH

Flashcard 419: What is the purpose of converting cholesterol into cholesterol ester? _____

Answer: More space-efficient (anhydrous)

Flashcard 420: What is the precursor molecule of thromboxane A2?_____

Answer: Arachidonic acid

Flashcard 421: What is the main component of LDLs?_____

Answer: Cholesterol ester

Flashcard 422: The triglycerides inside the VLDL are broken down into _____ by lipoprotein lipase in the vicinity of the adipocytes (or peripheral tissue)

Answer: fatty acids

Flashcard 423: What substrate accumulates in the lysosomes of a patient with Gaucher disease? _____

Answer: Glucocerebroside

Flashcard 424: Which lipoproteins (2) carry the most cholesterol? _____

Answer: LDL and HDL

Flashcard 425: Once in the vicinity of the liver, the IDL may directly bind a _____ receptor via apoprotein B-100

Answer: LDL

Flashcard 426: Oxidized LDL has a defect in its single apoB-100 and is no longer recognized; consequently, it is consumed by _____

Answer: macrophages

Flashcard 427: Cholestatic DrugsThe rate limiting step of bile acid synthesis is done via _____

Answer: cholesterol 7a-hydroxylase

Flashcard 428: Which lipoprotein is secreted by intestinal epithelial cells and delivers dietary TGs to peripheral tissue? _____

Answer: Chylomicrons

Flashcard 429: The main component of chylomicrons is _____

Answer: triglycerides

Flashcard 430: In adipose tissue, triglycerides are broken down into glycerol and fatty acids by the enzyme _____

Answer: hormone sensitive lipase

Flashcard 431: All steroid hormones are derivatives of _____

Answer: cholesterol

Flashcard 432: What form of estrogen is produced in the ovary?_____

Answer: 17-estradiol

Flashcard 433: Apoprotein E may be found on every lipoprotein except _____

Answer: LDL

Flashcard 434: The triglycerides inside the chylomicron are broken down into _____ by lipoprotein lipase in the vicinity of the adipocytes (or peripheral tissue)

Answer: fatty acids

Flashcard 435: _____ is an intermediate of cholesterol synthesis that is important for N glycosylation of proteins

Answer: Dolichol PPi

Flashcard 436: Which organ is responsible for the import and export of lipoproteins?_____

Answer: Liver

Flashcard 437: What are the two major ketone bodies produced in a type 1 diabetic patient?_____

Answer: -hydroxybutyrate and acetoacetate

Flashcard 438: Which adrenal cortex deficiency results in shunting towards the aldosterone synthesis pathway?_____

Answer: 17-hydroxylase deficiency

Flashcard 439: Where in the cell does cholesterol synthesis occur?_____

Answer: cytoplasm

Flashcard 440: Pulmonary surfactant is a mix of lecithins, the most important of which is _____

Answer: dipalmitoyl phosphatidylcholine (DPCC)

Flashcard 441: Which lipid transport enzyme is responsible for the degradation of remaining triglycerides in IDLs? _____

Answer: Hepatic TG lipase

Flashcard 442: Iron poisoning causes cell death due to _____ of membrane lipids

Answer: peroxidation

Flashcard 443: What demographic has an increased incidence of Tay-Sachs, Niemann Pick, and some forms of Gaucher disease?_____

Answer: Ashkenazi Jews

Flashcard 444: What is the main component of VLDLs?_____

Answer: Triglycerides

Flashcard 445: Which serum lipoprotein is elevated in type IV familial dyslipidemia? _____

Answer: VLDL

Flashcard 446: What substance is the precursor to bile acids, steroids, and vitamin D? _____

Answer: Cholesterol

Flashcard 447: Vitamin _____ injection is given at birth to prevent hemorrhagic disease of the newborn

Answer: K

Flashcard 448: Inside the intestinal epithelial cells, re-esterified lipids are packaged with apoproteins into _____

Answer: chylomicrons

Flashcard 449: The first intermediate in cholesterol synthesis is _____ (from HMG CoA)

Answer: mevalonate

Flashcard 450: Which lipoprotein is secreted by the liver and delivers hepatic TGs to peripheral tissue? _____

Answer: VLDL

Flashcard 451: Blood & Inflammation (Dyslipidemia drugs) _____ are the main source of cholesterol delivery to peripheral tissues in the body

Answer: LDLs

Flashcard 452: A ceramide attached to phosphate and choline is a _____

Answer: sphingomyelin

Flashcard 453: The bile salts and phospholipids in bile are _____ molecules, which allows for the emulsification and solubilization of lipids in an aqueous environment

Answer: amphipathic

Flashcard 454: Which two organs produce nascent HDL? _____

Answer: Liver, Intestines

Flashcard 455: Dyslipidemia drugs _____ deliver triglycerides from the intestine to peripheral tissues

Answer: Chylomicrons

Flashcard 456: Patients with Refsum disease do not die early as they can be treated with a _____ restricted diet and plasmapheresis

Answer: phytanic acid

Flashcard 457: Which enzymes (2) are unique to the zona fasciculata/reticularis and thus the glucocorticoid and androgen synthesis pathways?_____

Answer: 17-hydroxylase and 17,20-lyase

Flashcard 458: Dyslipidemia drugs _____ HDL particles contain LCAT-generated cholesterol esters

Answer: Mature

Flashcard 459: Oxidized LDL is consumed by macrophages via the _____ receptor

Answer: scavenger receptor-A

Flashcard 460: Are fatty acids polar or nonpolar?_____

Answer: Non-Polar

Flashcard 461: Apoprotein A-1 is a major component of _____ lipoprotein

Answer: high density

Flashcard 462: In Krabbe disease, galactocerebroside and psychosine accumulate in _____ and destroy the myelin sheath

Answer: macrophages

Flashcard 463: Which lipid transport enzyme is responsible for the degradation of triglycerides in the small intestine? _____

Answer: Pancreatic lipase

Flashcard 464: Which serum lipoprotein is elevated in type I hyperlipoproteinemia? _____

Answer: Chylomicrons

Flashcard 465: What is the rate-limiting enzyme for bile acid synthesis? _____

Answer: 7-hydroxylase

Flashcard 466: Which two phospholipids are abundant in surfactant?_____

Answer: lecithin (phosphatidylcholine) and sphingomyelin

Flashcard 467: Once inside the adipocyte, fatty acids are reconverted into _____

Answer: triglycerides

Flashcard 468: As triglycerides are removed from the chylomicron, the chylomicron shrinks, forming a _____

Answer: chylomicron remnant

Flashcard 469: Thromboxane A2 promotes platelet _____

Answer: aggregation

Flashcard 470: Which type of acyl-CoA dehydrogenase is used to breakdown 8-10 carbon fatty acyl-CoA?_____

Answer: Medium (medium chain acyl-CoA dehydrogenase)

Flashcard 471: The precipitated cholesterol in blood vessels may be picked up by _____ (lipoprotein) and esterified to cholesterol ester

Answer: HDL

Flashcard 472: Hormone sensitive lipase is regulated via negative feedback by _____

Answer: insulin

Flashcard 473: Cholesterol ester inside of mature HDL may be transferred to VLDL, IDL, or LDL via the enzyme _____

Answer: cholesterol ester transport protein (CETP)

Flashcard 474: Primary carnitine deficiency may present with _____-uria

Answer: myoglobin

Flashcard 475: Sulfatides are converted to galactocerebroside via the enzyme _____

Answer: arylsulfatase A

Keywords: Lipid metabolism flashcards, medical flashcards, NEET PG preparation, USMLE Step 1 flashcards, Anki alternative, spaced repetition medical, OnCourse flashcards

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Fatty acid oxidation (beta-oxidation)

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Fatty acid synthesis

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Ketone body metabolism

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Cholesterol synthesis and regulation

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Lipoprotein metabolism

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Phospholipid metabolism

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Eicosanoid synthesis and function

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Steroid hormone synthesis

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Adipose tissue metabolism

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Brown vs. white adipose tissue

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Disorders of lipid metabolism

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Integration with carbohydrate metabolism

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Lipid metabolism — Flashcards

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