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Biochem 2 > Glycogen > Flashcards

Glycogen Flashcards

exam 2

1
Q

where do you find glycogen?

A
  • liver

- muscle

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2
Q

starving hypoglycemia, elevated glycogen

A

GLUT2 deficiency

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3
Q

short-term glucose buffer

A

glycogen

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4
Q

High glucose would _____ proteins, pose a major _____ problem and would leak out due to _______ transport

A

High glucose would glycate proteins, pose a major osmotic problem and would leak out due to facilitated transport

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5
Q

First step of synthesis of glycogen

A

G6P—-> G1P by phsphoglucomutase

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6
Q

G1P needs to be activated.. how?

A

using UDP-glucose pyrophosphorylase to make UDP-Glucose

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7
Q

polymer synthesis involves UDP glucose and turning into

A

Glycogen alpha 1-4 by glycogen synthase

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8
Q

Glycogen alpha 1-4 can add

A

glycgoen alpha 1-6 via a branching enzyme

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9
Q

Glycogen synthase is limiting and a regulated step… why?

A

elongates by adding one glucose at a time starting with UDP-Glucose primer

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10
Q

Breakdown of glycogen involves glycogen phosphorylase which and the product is

A

cleaves one glucose at a time and the product is G1P

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11
Q

transfers three residues of a branching chain to a different chain

A

debranching enzyme

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12
Q

debranching enzyme cleaves the remaining glucose by ______ resulting in

A

debranching enzyme cleaves the remaining glucose by hydrolysis resulting in free glucose

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13
Q

muscle does not have this enzyme that is used in the breakdown of glycogen

A

glucose 6 phosphatase

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14
Q

maintain blood glucose concentration

A

insulin and glucagon

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15
Q

releases fuel when needed for stress response: via cyclic AMP and protein kinase A

A

epinephrine

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16
Q

chronically stimulates glycogenolysis

A

cortisol

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17
Q

release of fuel supply within muscle: via calcium release, calmodulin and protein kinase A

A

nerve-muscle stimulus

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18
Q

high glucose, G6P and G1) favors synthesis

A

dietary intake

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19
Q

inhibits glycogen breakdown

A

F1P

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20
Q

increased glycogen content slows synthesis

A

autoregulation

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21
Q

Gycogen phosphorylase is ________than P-Glycogen phosphorylase a

A

less active

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22
Q

Glycogen synthase is _____ than P-glycogen synthase

A

more active

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23
Q

P-glycogen phosphorylase a is induced by

A

glucagon

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24
Q

Glycogen synthase is induced by

A

insulin

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25
Q

in liver ______ stimulates glycogen breakdown

A

epinephrine

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26
Q

______ activates phosphorylase kinase

A

calcium

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27
Q

in muscle, ______ or _______ stimulates glycogen breakdown

A

epinephrine or nerve stimulation

28
Q

In a glucose tolerance test, a patient ingests a large amount of glucose. What will happen in a normal patient?

A. Inhibition of glycogen synthetase phosphatase
B. Increase rate of lactate formation by erythrocytes
C. Enhanced phosphorylase activity in the pancreas
D. Increased ratio of phosphorylase a to phosphorylase b
E. Enhanced glycogen synthase activity in liver

A

E. Enhanced glycogen synthase activity in liver

29
Q

Glucose-6-phosphatase defect

A

von Gierke disease

30
Q

von Gierke disease type 1B

A

transported defect

31
Q

Lysosomal alpha 1-4 glucosidase defect

A

Pompe disease

32
Q

Debranching enzyme defect

A

Cori’s disease

33
Q

Branching enzyme defect

A

Andersen’s disease

34
Q

A defect in which of the following enzymes would lead to glycogen with a defective structure (as opposed to a change in amount).

	A. 	adenyl cyclase
	B. 	 glycogen branching enzyme
	C. 	glycogen synthase
	D. 	phosphorylase kinase
	E.      phosphorylase.
A

B. glycogen branching enzyme

35
Q

A baby presents with growth retardation, hepatomegaly and hypoglycemia. Liver biopsy shows small dextrin-like molecules in the cytoplasm of cells. What is likely to be wrong?

	A. Type I GSD von Gierke
	B. Type II GSD Pompe
	C. Type III GSD Cori 
              disease
	D. Type IV GSD  
             Andersen
        E. Type V GSD McArdle
A

C. Type III GSD Cori

disease

36
Q

Skeletal muscle glycogen phosphorylase defect

A

McArdle disease

37
Q

Liver glycogen Phosphorylase defect

A

Hers disease

38
Q 
A college girl taking up organized sports for the first time complains of severe leg cramps after trying out for the soccer team.  She also noticed a reddish tinge in her urine that night.  You order an ischemic forearm exercise test that shows no lactate.  Where is the defect?
A. Glucose 6-phosphatase
B. Acid maltase
C. alpha 1-6 glucosidase
D. Glycogen phosphorylase
E. Cystathionine synthetase
A

D. Glycogen phosphorylase

39
Q

phosphofructokinase defect

A

Tauri’s disease

40
Q

excess glucose stored as

A

glycogen

41
Q

Glucose is converted to ______ then into ______.

A

Glucose is converted to G6P then into G1P

42
Q

Where is glycogen found?

A

liver and muscles

43
Q

there is a greater amount of glycogen in _____… why?

A

muscles because there are more muscles than liver

44
Q

why make glycogen? (4)

A
  1. glucose can leak out
  2. osmotic pressure problem
  3. glycation causes tissue damage- glucose causes it
  4. easier regulation
45
Q

enzyme that converts G6P to G1P

A

phosphoglucomutase

46
Q

which enzyme adds UTP to GIP?

A

UDP- Glucose pyrophsphorylase activates glucose

47
Q

which is the limiting/regulating step in glycogen synthesis?

A

polymer synthesis- UDP-glucose to glycogen

48
Q

glycogen synthase requires a

A

primer- usually an existing glycogen strand

49
Q

if no glycogen exists, then the enzyme ______ will act as a primer adding UDP-Glucose on _____

A

if no glycogen exists, then the enzyme Glycogenin will act as a primer adding UDP-Glucose on Tyr 194

50
Q

branching allows for

A

faster mobilization of glycogen

51
Q

enzyme that breaks down glycogen

A

glycogen phosphorylase

52
Q

Glycogen phosporylase is the ________ step and will use ____ to cleave glycogen and create G1P as a product.

A

Glycogen phosporylase is the limiting step and will use Pi to cleave glycogen and create G1P as a product.

53
Q

debranching enzyme has both ____ and _____ activity

A

transferase and hydrolase

54
Q

strongest promoter of glycolysis

A

F26BP

55
Q

muscle glycogen breakdone results in _____ but not in the liver

A

glycolysis

56
Q

Glycogen phosphorylase is activated by

A

AMP and calcium

57
Q

Glycogen phosphorylase is inhibited by

A

ATP and G6P

58
Q

phosphorylase a is in the

A

active form

59
Q

phosphorylase b is in the

A

inactive form

60
Q

______ will cause the formation of cAMP which will activate phosphorylase kinase leading to ____

A

Glucagon leads to glycogen breakdown

61
Q

Insulin will _____________ glycogen phosphorylase leading to its _______ and activating

A

Insulin will dephosphorylate leading to its inactivation and activating glycogen synthesis

62
Q

epinephrine leads to

A

glycogen breakdown

63
Q

pompe disease leads to

A

cardiomegaly

64
Q

fatal due to the altered structure of glycogen

A

Anderson’s

65
Q

red tinge urine

A

mcArdle

66
Q

normal glycogen structure but altered amount

A

Hers disease

67
Q

fructose helps

A

Tauri’s disease

Biochem 2 (14 decks)

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