Glycogen Metabolism Flashcards

1
Q

How is glycogen stored?

A

Large cytosolic granules

10% of liver mass and 1-2% of muscle mass

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2
Q

What is the structure of glycogen?

A

a1->4 linked glucose with a1->6 branches

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3
Q

Where does the addition and removal of glucose in glycogen occur?

A

The non-reducing ends

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4
Q

What is the function of glycogen phosphorylase and what cofactor does it require?

A

Removes glucose from non-reducing ends until it reaches four glucose molecules from a branch point

Requires pyridoxal phosphate

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5
Q

What is the function of glycogen debranching enzyme?

A

Has two activities:

Transferase activity moves 3 glucose moelcules from branch to non-reducing end

Glucosidase activity removes remaining glucose molecule

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6
Q

What is the function of phosphoglucomutase?

A

Converts glucose-1-phosphate converted to glucose-6-phosphate

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7
Q

What is the function of glucose 6-phosphatase?

A

Converts G6P to glucose.

Phosphatase is expressed in the liver and kidney, but not muscle and adipose tissue

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8
Q

Where does glycogen synthesis take place?

A

Liver and Skeletal muscle

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9
Q

What is the first step in glycogen synthesis?

A

G6P to G1P via phosphoglucomutase

Reversal of reaction in glycogen breakdown

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10
Q

What is the function of UDP-glucose pyrophosphorylase?

A

Converts G1P to UDP-glucose

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11
Q

What is the function of glycogen synthase?

A

Adds glucose residue of UDP-glucose to the non-reducing end of glycogen

Removes UDP

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12
Q

What is the function of glycogen branching enzyme?

A

Adds a1–>6 branches

Removes 7 residues, 3 residues down to form a branch

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13
Q

What two things does the initiation of a new glycogen chain require?

A

Primer and glycogenin

Primer is usually a preformed polyglucose chain

Glycogenin can act as both a primer and enzyme

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14
Q

What is the function of glycogenin?

A

Binds to UDP-glucose and removes UDP. This continues six more times through the addition of glucose and removal of UDP.

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15
Q

Describe the structure of glycogen phosphorylase

A

Made up of two subunits, each exists in an a and b form

a = catalyticall active
b = less active
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16
Q

How is glycogen phosphorylase regulated?

A

By phosphorylation.

Phosphorylated = a form, active

Not Phosphorylated = b form, less active

17
Q

What is the function of phosphorylase b kinase?

A

To phosphorylate glycogen phosphorylase b and activated it.

18
Q

How is phosphorylase b kinase activated in the muscle?

A

epinephrine, increased Ca, increased AMP

19
Q

How is phosphorylase b kinase activated in the liver?

A

Glucagon

20
Q

What inhibits phosphorylase b kinase?

A

ATP, G6P, and insulin

Stimulate dephosphorylation via phosphatase

21
Q

What is the function of Phosphoprotein phosphatase 1 (PP1)?

A

Dephosphorylates glycogen phosphorylase a

22
Q

What signaling pathway initiates glycogen breakdown?

A

cAMP, PKA

23
Q

How does glucose concentration affect phosphorylase activity?

A

Phosphorylase can act as a glucose sensor. Glucose can allosterically inactivate phosphorylase a and promote dephosphorylation

24
Q

How is glycogen synthase regulated?

A

Phosphorylation

a form, active - not phosphorylated

b form, inactive, - phosphorylated

25
Q

How is the dephosphorylation of glycogen synthase stimulated?

A

Insulin, G6P, glucose

26
Q

What stimulates the phosphorylation of glycogen synthase?

A

Glucagon and epinephrine

27
Q

What are three regulations of carbohydrate metabolsim specific to muscle?

A

Lack enzymatic machinery for gluconeogenesis

Lack receptors for glucagon

Isozyme of pyruvate kinase not phosphorylated by PKA
-so glycolysis is not turned off when cAMP is high

28
Q

How do glycogen storage diseases present?

A

Hypoglycemia

Liver damage and enlargement

Myopathy