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Drew 922 > Block 3 Lecture 5 -- Formation and Degradation of Glycogen > Flashcards

Block 3 Lecture 5 -- Formation and Degradation of Glycogen Flashcards

1
Q

glycogen branches every how many residues

A

8-14

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2
Q

What does the reducing end of glycogen contain

A

the 1 free anomeric C

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3
Q

What is the branching linkage

A

alpha1-6

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4
Q

How is glycogen stored

A

as granules

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5
Q

What are the enzymes of glycogenolysis?

A

1) GPase
2) Debranching
3) PGM
also G6Pase but only in liver

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6
Q

What are the enzymes of glycogen synthesis?

A

1) UDP-Glucose Pyrophosphorylase
2) Glycogen Synthase
3) Branching Enzyme
glycogenin is the primin enzyme

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7
Q

Which form of GPase is active?

A

PO4

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8
Q

Which form of GS is active?

A

not phosphorylated

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9
Q

Which form of GPK is active?

A

PO4

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10
Q

2 forms of GPase?

A

a and b; a is active

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11
Q

GPase action?

A

generates G1P one at a time from non-reducing end except on short 4-residue branches; requires PLP

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12
Q

What is the RLS is glycogen breakdown?

A

GPase

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13
Q

Regulation of GPase?

A

a/b interconversion

allosterics: ATP, G6P, glucose inhibit; AMP activates

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14
Q

Describe action of debranching?

A

trisaccharide transfer and hydrolysis of remaining residue

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15
Q

What is the action of PGM?

A

G1P –> G6P or reverse

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16
Q

What is action of UDP-glucose pyrophosphorylase?

A

G1P + UTP –> UDP-glucose + PPi

17
Q

What is activity of glycogen synthase?

A

adds glucose from UDP-glucose to nonreducing end as 1-4 via Sn1

18
Q

Activity of branching enzyme?

A

cleaves 6-8 residues from 11-13 residue chain and attaches them 1-6

19
Q

Action of glycogenin?

A

glycosylated by tyr glycosyltransferase, then adds up to 7 more glucose units using UDP-glucose

20
Q

Deficient enzyme in Type 1?

A

liver G6Pase

21
Q

What is type 1

A

von Gierkes

22
Q

Deficient enzyme in Coris?

A

debranching

23
Q

Deficient enzyme in Type IV?

A

Andersen’s

24
Q

Deficient enzyme in Type V

A

muscle GPase

25
Q

Deficient enzyme in Type VI?

A

liver Gpase

26
Q

Deficient enzyme in Type VII?

A

Muscle PFK

27
Q

What is type 7?

A

taruis

28
Q

What is type 6?

A

hers’

29
Q

What is type 5?

A

mcardle’s

30
Q

What is type 4

A

andersens

31
Q

What is type 3?

A

coris

32
Q

What is the most lethal form of glycogen storage diseases?

A

type 2 / pomepe’s

Drew 922 (12 decks)

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