Glycogen Metabolism Flashcards

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1
Q

What enzyme converts G6P to G1P?

A

Phosphoglucomutase

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2
Q

What enzyme activates glucose to form UDP-Glucose?

A

G1P UTP uridylyltransferase

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3
Q

What is glycogen branching enzyme?

A

A1;4,a1-6 Tranferase

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4
Q

Which end of the glycogen chain is attached to glycogenin?

A

Reducing end

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5
Q

What is the role of glycogen phosphorylase?

A

Phosphorylates and cleaves G1P from glycogen

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6
Q

What is debranching enzyme?

A

A1-4, a1-4 transferase

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7
Q

What enzyme removes the last glucose from a glycogen side chain?

A

Glucosidase

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8
Q

Describe Von Gierke Disease.

A
  • Lack of G6Phospatase In ER Lumen

- Also could be protein which transports G6P from cytosol to ER

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9
Q

What enzyme is deficient in Pompe’s disease?

A

Lysosomal a-glucosidase

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10
Q

What enzyme is deficient in McArdles disease?

A

Skeletal muscle phosphorylase

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11
Q

What enzyme is deficient in Cory’s disease?

A

DeBranching enzyme

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12
Q

What enzyme is deficient in Andersons disease?

A

Defect in Branching enzyme

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13
Q

What enzyme is deficient in Hers disease?

A

Liver phosphorylase deficiency

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14
Q

What are the three key features which distinguish Diabetes vs Hyperlipidemia?

A
  • Insulin/Glucagon levels
  • Adipose tissue Lipase C not activated?
  • Triglyceride accumulation in serum?
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