Carbohydrate Metabolism — Flashcards

Flashcard 1: Which steps (2) of glycolysis require ATP? _____

Answer:

Flashcard 2: _____ is the glycosaminoglycan present in the granules of the given mononuclear cell / mast cell.

Answer: Heparin

Flashcard 3: Which steps (2) of glycolysis produce ATP? _____

Answer:

Flashcard 4: breast millk is richest in _____ carbohydrate

Answer: lactose

Extra: 

Flashcard 5: Cori disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: functional

Flashcard 6: _____ or mucopolysaccharides are complex carbohydrates made up of amino sugars and uronic acids

Answer: Glycosaminoglycans

Flashcard 7: Treatment of Von Gierke disease includes avoidance of _____, galactose, sucrose, and lactose

Answer: fructose

Flashcard 8: _____ is characterized by the absence of the Gal-1-P uridyltransferase enzyme

Answer: Classic galactosemia

Flashcard 9: Glycogen synthase is regulated via negative feedback by _____ and glucagon

Answer: epinephrine

Flashcard 10: PEP carboxykinase is regulated via positive feedback by _____ and cortisol

Answer: glucagon

Flashcard 11: _____ disease is also known as glycogen storage disease type VI

Answer: Hers

Flashcard 12: GLUT _____ helps in the insulin dependent uptake of glucose by the blastocyst cells

Answer: 8

Flashcard 13: _____ disease is also known as glycogen storage disease type III

Answer: Cori

Flashcard 14: Glycogen stores typically are depleted after _____ - 18 hours

Answer: 12

Flashcard 15: Once inside the pancreatic -cell, glucose is _____ by the enzyme glucokinase

Answer: phosphorylated

Flashcard 16: _____ disease is caused by a deficiency of the enzyme debranching enzyme (-1,6-glucosidase)

Answer: Cori

Flashcard 17: Von Gierke disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: impaired

Flashcard 18: The tissues/organs that have both aldose reductase and sorbitol dehydrogenase include the _____, ovaries, and seminal vesicles

Answer: liver

Flashcard 19: _____ disease is caused by a deficiency of the enzyme muscle glycogen phosphorylase (myophosphorylase)

Answer: McArdle

Flashcard 20: One function of the HMP shunt is to provide a source of _____, which is used for nucleotide synthesis and glycolytic intermediates

Answer: ribose

Flashcard 21: What substrates (2) accumulate in the lysosomes of a patient with Hunter syndrome? _____ and dermatan sulfate

Answer: Heparan sulfate

Flashcard 22: _____ disease is also known as glycogen storage disease type I

Answer: Von Gierke

Flashcard 23: _____ disease is also known as glycogen storage disease type V

Answer: McArdle

Flashcard 24: The tissues/organs that have only aldose reductase (no sorbitol dehydrogenase) may be remembered with the mnemonic "LuRKS":L: _____uR: RetinaK: KidneysS: Schwann cells

Answer: Lens (primarily aldose reductase)

Flashcard 25: The sites of synthesis of glycosaminoglycans (GAG) are _____ and golgi apparatus.

Answer: endoplasmic reticulum

Flashcard 26: _____ may be converted to phosphoenolpyruvate via the enzyme PEP carboxykinase

Answer: Oxaloacetate

Flashcard 27: Von Gierke disease is characterized by very increased levels of _____ in the liver and kidneys, with subsequent hepatomegaly and renomegaly

Answer: glycogen

Flashcard 28: _____ disease is also known as glycogen storage disease type IV

Answer: Andersen

Flashcard 29: Fructose-1,6-bisphosphatase is regulated via negative feedback by _____ and AMP

Answer: fructose-2,6-bisphosphate

Flashcard 30: Insulin decreases blood glucose concentration by promoting synthesis and storage of _____ in the muscle and liver, while simultaneously inhibiting glycogenolysis

Answer: glycogen

Flashcard 31: _____ disease is also known as glycogen storage disease type II

Answer: Pompe

Flashcard 32: Von Gierke disease is associated with increased blood _____ate, triglycerides, and uric acid

Answer: lact

Flashcard 33: When insulin acts on liver tissue, it causes _____ glycogenesis and decreased glycogenolysis

Answer: increased

Flashcard 34: What symptoms does essential fructosuria typically present with?_____

Answer: Asymptomatic

Flashcard 35: _____ results in the formation of -(1,6) bonds between glucose molecules

Answer: Branching enzyme

Flashcard 36: _____ disease occurs due to a defect in the enzyme muscle phosphofructokinase.

Answer: Tauri's

Flashcard 37: _____ liberates glucose-1-phosphate residues off branched glycogen until 4 glucose units remain on a branch

Answer: Glycogen phosphorylase

Flashcard 38: _____ is ultimately converted to ribulose-5-phosphate via the enzyme glucose-6-phosphate dehydrogenase

Answer: Glucose-6-phosphate

Flashcard 39: Glucose-6-phosphate is converted to _____ via the enzyme glucose-6-phosphatase

Answer: glucose

Flashcard 40: Large amounts of _____ are given for treatment of abetalipoproteinemia so that excess will be converted to short-chain fatty acids in the colon.

Answer: carbohydrates

Flashcard 41: Pyruvate may be converted to _____ via the enzyme pyruvate carboxylase

Answer: oxaloacetate

Flashcard 42: Glucose is converted to _____ by the enzyme hexokinase in most tissues

Answer: glucose-6-phosphate

Flashcard 43: The branched glycogen molecule (pre-debranching enzyme) is cleaved by the enzyme _____ in the lysosomes, only

Answer: -1,4-glucosidase (acid maltase)

Flashcard 44: Pyruvate is converted to _____ via the enzyme lactate dehydrogenase (LDH) (Cori cycle)

Answer: lactate

Flashcard 45: Pyruvate carboxylase requires _____, biotin, and CO2

Answer: ATP

Flashcard 46: When _____ glucose unit remains on a branch of glycogen, debranching enzyme (-1,6-glucosidase) cleaves off the last residue, liberating glucose

Answer: one

Flashcard 47: _____ is converted to UDP-glucose via the enzyme UDP-glucose pyrophosphorylase (glycogen synthesis)

Answer: Glucose-1-phosphate

Flashcard 48: _____ disease is caused by a deficiency of the enzyme glucose-6-phosphatase

Answer: Von Gierke

Flashcard 49: Gluconeogenesis occurs primarily in the _____, but necessary enzymes are also found in the kidney and intestinal epithelium

Answer: liver

Flashcard 50: _____ is converted into pyruvate via the enzyme pyruvate kinase

Answer: Phosphoenolpyruvate

Flashcard 51: Glucagon increases blood glucose concentration by increasing _____, while simultaneously inhibiting glycogen formation from glucose

Answer: glycogenolysis

Flashcard 52: The byproducts of _____ fermentation are gases (CO2, H2) and short chain fatty acids (ie: lactic acid)

Answer: anaerobic

Flashcard 53: Glycogen synthase is regulated via positive feedback by _____, cortisol, and glucose-6-phosphate

Answer: insulin

Flashcard 54: _____ is converted into galactose and glucose by the enzyme lactase (intestinal brush border)

Answer: Lactose

Flashcard 55: _____ is converted to fructose-2,6-bisphosphate via the enzyme phosphofructokinase-2 (PFK-2)

Answer: Fructose-6-phosphate

Flashcard 56: Glucocorticoids are a potent stimulator for _____, thus they are essential for survival during fasting

Answer: gluconeogenesis

Flashcard 57: Galactose is converted to _____ via the enzyme galactokinase

Answer: galactose-1-phosphate

Flashcard 58: Reducing disaccharides include _____ and maltose

Answer: lactose

Flashcard 59: Pyruvate dehydrogenase is regulated via negative feedback by _____, NADH, and acetyl-CoA

Answer: ATP

Flashcard 60: When _____ glucose units remain on a branch of glycogen, debranching enzyme (4--D-glucanotransferase) moves 3 molecules of glucose-1-phosphate from the branch to the linkage

Answer: four

Flashcard 61: Phosphofructosekinase-1 is regulated via positive feedback by _____ and fructose-2,6-bisphospate

Answer: AMP

Flashcard 62: _____ is converted to sorbitol via the enzyme aldose reductase

Answer: Glucose

Flashcard 63: _____ is converted to fructose-6-phosphate via the enzyme isomerase

Answer: Glucose-6-phosphate

Flashcard 64: Fructose is converted into _____ by the enzyme fructokinase

Answer: fructose-1-phosphate

Flashcard 65: HMP shunt activity is primarily found in the _____ glands, liver, lactating mammary glands (females), and RBCs

Answer: adrenal

Flashcard 66: Trehalose is a disaccharide that is digested into _____ and glucose by trehalase

Answer: glucose

Flashcard 67: Increased phosphofructokinase-2 _____ glycolysis and inhibits gluconeogenesis

Answer: stimulates

Flashcard 68: The respiratory quotient, if someone is on an exclusive _____ diet, is 1.

Answer: carbohydrate

Flashcard 69: _____ is converted to glyceraldehyde and dihydroxyacetone-P via the enzyme aldolase B *primarily in the liver

Answer: Fructose-1-phosphate

Flashcard 70: One hallmark of cancer is the Warburg effect, in which cancer cells shift glucose metabolism from _____ toward glycolysis

Answer: mitochondria

Flashcard 71: In diabetes patients, _____ damage may occur when glucose in tissue is converted to sorbitol by the enzyme aldose reductase

Answer: osmotic

Flashcard 72: _____ may be converted to fructose-6-phosphate via the enzyme fructose-1,6-bisphosphatase

Answer: Fructose-1,6-bisphosphate

Flashcard 73: The enzyme _____ catalyzes the formation of -(1,4) bonds between glucose molecules

Answer: glycogen synthase

Flashcard 74: Glycogen phosphorylase is regulated via negative feedback by _____, insulin, and glucose-6-phosphate

Answer: ATP

Flashcard 75: Glucose is converted to _____ by the enzyme glucokinase in the liver and cells of the pancreas

Answer: glucose-6-phosphate

Flashcard 76: Galactose-1-phosphate is ultimately converted to _____ via the enzyme Gal 1-P uridyltransferase

Answer: glucose-1-phosphate

Flashcard 77: Increased insulin (fed) leads to decreased cAMP and protein kinase A, thus _____ activity of FBPase-2 and increasing activity of PFK-2

Answer: decreasing

Flashcard 78: Fructose-1,6-bisphosphatase is regulated via positive feedback by _____ and ATP

Answer: citrate

Flashcard 79: Tauri's disease presents similar to McArdle's with additional _____ and growth retardation

Answer: hemolytic anemia

Flashcard 80: Glycogen phosphorylase is regulated by positive feedback by _____ via the beta receptor (liver only)

Answer: epinephrine

Flashcard 81: Hers disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: functional

Flashcard 82: Deficiency of _____ or xylitol dehydrogenase necessary for xylitol metabolism results in Pentosuira

Answer: L-xylulose reductase

Flashcard 83: UDP-glucose contributes to _____ formation via the enzyme glycogen synthase

Answer: glycogen

Flashcard 84: _____ is converted to fructose-1,6-bisphosphate via the enzyme phosphofructokinase-1 (PFK-1)

Answer: Fructose-6-phosphate

Flashcard 85: _____ is converted to fructose via the enzyme sorbitol dehydrogenase

Answer: Sorbitol

Flashcard 86: Isomers that differ in configuration around only one specific carbon atom (with the exception of the carbonyl carbon) are defined as _____ of each other.

Answer: epimers

Flashcard 87: _____ disease is caused by a deficiency of the enzyme hepatic glycogen phosphorylase

Answer: Hers

Flashcard 88: Which GAG is exclusively intracellular?_____

Answer: Heparin

Flashcard 89: Which GAG has no sulfate group?_____

Answer: Hyaluronic acid

Flashcard 90: What are the 3 enzymes catalyzing irreversible reactions in the glycolytic pathway?_____

Answer: Hexokinase, PFK-1 and Pyruvate kinase (3)

Flashcard 91: What is the most potent allosteric activator of PFK-1?_____

Answer: Fructose 2,6 bisphosphate

Flashcard 92: _____ differ in the orientation of the  H and  OH groups around the carbon atom adjacent to the terminal alcohol carbon

Answer: Enantiomers

Flashcard 93: A pool of GLUT-4 molecules is maintained within vesicles in the _____ of insulin-sensitive cells.

Answer: cytoplasm

Flashcard 94: Which GAGs do NOT contain uronic acid?_____

Answer: Keratan sulfate

Flashcard 95: What is the only GAG that is not synthesized by the golgi apparatus?_____

Answer: Hyaluronic acid

Flashcard 96: What protein is the primer acting as an acceptor of glucose residues in the process of glycogen synthesis?_____

Answer: Glycogenin

Flashcard 97: What is the most abundant GAG in the body?_____

Answer: Chondroitin sulfate

Flashcard 98: Breakdown of glycogen under anaerobic conditions yields _____ ATPs.

Answer: 3

Flashcard 99: Which glucose transport receptors are reduced after overnight fasting?_____

Answer: GLUT-4

Flashcard 100: In refeeding syndrome, severe _____glycemia is seen after excessive insulin release

Answer: hyper

Flashcard 101: _____ forms rhombic plate-shaped crystals in osazone test

Answer: Galactose

Flashcard 102: _____ differ in the orientation of the  H and  OH groups around the 1st carbon atom in the ring structure of carbohydrates

Answer: Anomers

Flashcard 103: D-Glucose and D-mannose are examples of _____-epimers

Answer: C2 (carbon number)

Flashcard 104: De Vivo disease is a metabolic disorder due to _____ deficiency.

Answer: GLUT1

Flashcard 105: Glucose-6-phosphatase is a marker for _____

Answer: endoplamic reticulum (cell organelle)

Flashcard 106: The percentage of _____ in human milk is greater than that in cow s milk.

Answer: lactose

Flashcard 107: What is the first committed step of glycoysis?_____

Answer: Fructose 6 phosphate to Fructose 1, 6 bisphosphate

Flashcard 108: Glycemic index is highest for _____.

Answer: glucose

Flashcard 109: _____ forms broomstick/ needle-shaped crystals in osazone test

Answer: Glucose, Fructose and Mannose (3)

Flashcard 110: Majority of the energy requirement in the lens is met by _____

Answer: glycolysis

Flashcard 111: Essential pentosuria is a benign condition caused due to a defect in the _____ pathway.

Answer: uronic acid

Flashcard 112: D-Glucose and D-galactose are examples of _____-epimers

Answer: C4 (carbon number)

Flashcard 113: Pancreatic amylase acts on _____ alpha 1,4 glycosidic linkage

Answer: internal (external/internal)

Flashcard 114: What is the highest energy organic compund?_____

Answer: Phosphoenolpyruvate

Flashcard 115: Increased accumulation of sorbitol in lens in diabeties leads to secondary _____

Answer: osmotic overhydration (not cataract)

Flashcard 116: _____ forms petal-shaped / sunflower-shaped crystals in osazone test

Answer: Maltose

Flashcard 117: Which enzymes are involved in the non-oxidative phase of HMP shunt pathway?_____

Answer: Transketolase and Transaldolase (2)

Flashcard 118: _____ forms puff-shaped crystals in osazone test

Answer: Lactose

Flashcard 119: What element is the co-factor for PFK-1 enzyme of glycolysis?_____

Answer: Magnesium

Flashcard 120: Which step of glycolysis utilizes an inorganic phosphate?_____

Answer: glyceraldehyde-3-phosphate to 1,3 biphosphoglycerate

Flashcard 121: _____ is the only cellular substrate that can be phosphorylated by glucokinase.

Answer: Glucose

Flashcard 122: _____ is the glycosaminoglycan present in the granules of the given mononuclear cell / mast cell.

Answer: Heparin

Flashcard 123: _____ galactosemia is caused to deficient galactose-1-phosphate uridyl transferase.

Answer: Classical

Flashcard 124: The source of blood glucose in a person fasting overnight is hepatic _____.

Answer: glycogenolysis