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Basic Biochemistry > 41. Glycogen Metabolism > Flashcards

41. Glycogen Metabolism Flashcards

1
Q

major storage cacrbohydrate in animals

A

glycogen

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2
Q

______ gram of glycogen

stored in muscle and liver

A

500 grams

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3
Q

where does glycogenesis occur

A
  • liver and muscle

* cytosol

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4
Q

rate limiting step of glycogenesis

A

elongation of glycogen chains
alpha (1,4) glycosidic bonds
enzyme: glycogen synthase

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5
Q

protein that serves as a primer for glycogen synthesis when glycogen is completely depleted

A

glycogenin

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6
Q

synthesis of UDP-glucose requires this enzymes

A

phosphoglucomutase

UDP-glucose pyrophosphorylase

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7
Q

source of all glucosyl residues that are added to glycogen molecule

A

UDP-glucose

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8
Q

elongation of glycogen chains

enzyme

A

glycogen synthase

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9
Q

rate limiting step of glycogenesis

A

elongation of glycogen chain
•glycogen synthase
•bonds formed at non-reducing end (C4)

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10
Q

formation of branches in glycogen

enzyme ?

A

branching enzyme

•amylo alpha (1–>4) –> alpha (1–>6) transglucosidase

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11
Q

forms alpha (1–>6) bonds by transferring 5-8 glucosyl residues

A

branching enzyme

•amylo alpha (1–>4) –> alpha (1–>6) transglucosidase

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12
Q

forms alpha (1–>4) bonds between glucose residues

A

glycogen synthase

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13
Q

for mobilizing stored glycogen

A

glycogenolysis

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14
Q

where does glycogenolysis occur

A
  • liver, muscle

* cytosol

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15
Q

what is the substrate of glycogenolysis

A

glycogen

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16
Q

what are products of glycogenolysis

A
  • glucose - liver

* glucose-6-phosphate - muscle

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17
Q

rate limiting step of glycogenolysis

A

shortening of glycogen chains
enzyme : glycogen phosphorylase
- cleavage of alpha (1–>4) bonds
between glucosyl residues at non reducing ends of glycogen chains

co-enzyme: pyridoxal phosphate

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18
Q

when does shortening of glycogen chain stop

A

when 4 glucosyl units remain (limit dextrin)

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19
Q

removal of branches of glycogen

enzyme?

A

debranching enzyme :
•alpha (1–>4) -> alpha (1–>4) glucantransferae
•amylo-alpha (1–>6) glucosidase

  • cleaves alpha (1–>4) and alpha (1–>6) bonds
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20
Q

debranchign enzyme yields free glucose from cleavage of ___ bond

A

alpha (1–>6) bonds

21
Q

conversion of glucose-1-P to glucose -6-P or glucose

enzyme

A

phosphoglucomutase
and when present
glucose-6-phosphatase

22
Q

lysosomal degradation of glycogen

A

alpha (1–>4) glucosidase (acid maltase)

23
Q

degrades 1-3% of glycogen

A

lysosomal degradation of glycogen

24
Q

deficiency of this enzyme leads to Pompe disease

A

alpha (1–>4) glucosidase (acid maltase)

25
Q

glycogen synthase

pathway

A

glycogenesis

26
Q

glycogen phosphorylase

pathway

A

glycogenolysis

27
Q

Presence of glucose-6-P
effect on
glycogen synthase

A

activated

28
Q

Presence of glucose-6-P
effect on
glycogen phosphorylase

A

inhibited

29
Q

Presence of ATP
effect on
glycogen phosphorylase

A

inhibited

30
Q

Presence of calcium (muscle)
effect on
glycogen phosphorylase

A

activated

31
Q

Presence of glucagon and epinephrine
on
glycogen synthase

A

inhibited

32
Q

Presence of insulin
effect on
glycogen synthase

A

activated

33
Q

phosphorylation

effect on
glycogen synthase

A

inactive

34
Q

Presence of glucagon and epinephrine

effect on
glycogen phosphorylase

A

activated

35
Q

Presence of INSULIN
effect on
glycogen phosphorylase

A

Inhibited

36
Q

PHOSPHORYLATION
effect on
glycogen phosphorylase

A

Active

37
Q

group of inherited disorders characterized by deposition of abnormal type or quantity of glycogen in tissues

A

glycogen storage diseases

38
Q

glucose-6-phosphatase deficiency

A

Type Ia

Von Gierke disease

39
Q

lysosomal acid maltase deficiency

A

Type II Pompe disease

40
Q

debranching enzyme deficiency

A

Type III a cori disease

41
Q

branching enzyme deficiency

A

type IV Andersen disease

42
Q

muscle phosphorylase deficiency

A

Type V McArdle syndrome

43
Q

liver phosphorylase deficiency

A

Type VI

Hers disease

44
Q
  • ÎÎ glycogen in liver and renal tubule cells
  • hypoglycemia
  • lactic acidosis
  • ketosis
  • hyperlipidemia
A

Von Gierke disease

45
Q
  • ÎÎ glycogen in lysosomes
  • Juvenile onset - hypotonia, death, heart failure
  • adult onset - muscle dystrophy
A

Pompe disease

46
Q
  • fasting hypoglycemia , hepatomegaly in infancy
  • ÎÎ limit dextrin
  • muscle weakness
A

Type IIIa cori disease

47
Q
  • hepatosplenomegaly
  • ÎÎ polysaccharide with few branch points
  • death from heart or liver failure before age 5
A

Anderrsen disease

48
Q
  • poor exercise tolerance
  • muscle cramps and myoglobinuria but no alctic acidosis
  • ÎÎ muscle glycogen
A

McArdle syndroe

49
Q
  • Hepatomegaly

* mild hypoglycemia

A

Hers disease

Basic Biochemistry (50 decks)

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