Glycogen storage diseases US Medical PG Flashcards - Medical Study Cards
Master Glycogen storage diseases 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.
Glycogen storage diseases Flashcard Deck - 13 Cards
Flashcard 1: glycogen synthesis
Answer: glycogen synthase
Flashcard 2: glycogenolysis
Answer: glycogen phosphorylase
Flashcard 3: McArdle's disease (GSD type V)
Answer:
Extra: painful muscle cramps, myoglobinuria with strenuous exercise glycogen accumulation in muscle AR skeletal muscle glycogen phosphorylase mnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)
Flashcard 4: Cori's disease (GSD type III)
Answer:
Extra: hepatomegaly fasting hypoglycemia, increased hepatic glycogen stores, normal blood lactate levels AR glycogen debranching enzyme mnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)
Flashcard 5: von Gierke's disease (GSD type I)
Answer:
Extra: hepatomegaly severe fasting hypoglycemia, increased blood lactate very increased hepatic glycogen stores AR glucose-6-phosphatase mnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)
Flashcard 6: Describe, in general, the glycogen storage diseases.
Answer: Diverse group of diseases resulting from defects in glycogen metabolism, characterized by an accumulation of glycogen within cells.
Flashcard 7: Describe the glycogen molecule structure.
Answer: Large, branched polymer of glucose
Flashcard 8: What are the main sites of glycogen storage?
Answer: liver, muscle
Flashcard 9: What type of bonds are found at glycogen linkages?
Answer: α (1,4) bonds
Flashcard 10: What type of bonds are found at glycogen branches?
Answer: α (1,6) bonds
Flashcard 11: glycogen phosphorylase
Answer: glycogenolysis
Extra: AMP, epinephrine, glucagon ATP, insulin
Flashcard 12: glycogen synthase
Answer: glycogen synthesis
Extra: glucose, insulin epinephrine, glucagon
Flashcard 13: If an enyzme is a phosphorylase, what do you know about its function?
Answer: adds phosphate to substrate WITHOUT use of ATP
mnemonic: -tase takes away
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