1: Glycogen Metabolism Regulation Flashcards

1
Q

When are glycogen synthase and glycogen phosphorylase active/inactive?

A

GS: active dephospho
GP: active phospho

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2
Q

When are glycogenesis and glycogenolysis favored?

A

Glycogenesis: fed state
Glycogenolysis: fasting state and during exercise

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3
Q

Three things that trigger glycogenesis

A

Blood glucose, insulin, ATP

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4
Q

Four things that trigger glycogenolysis

A

Glucagon, Ca in exercising muscles, AMP, low blood glucose

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5
Q

G6P effect on GS and GP

A

Stimulates GS, inhibits GP

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6
Q

Free glucose effect on GP

A

Inhibits GP in liver but not in muscle

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7
Q

Major effects of glycogen storage disorders that affect breakdown vs synthesis

A

Affect breakdown -> hepatomegaly, hypoglycemia

Affect synthesis -> dependent on glucose rather than glycogen

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8
Q

GSD 0 deficiency

A

GS (glycogen synthase)

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9
Q

GSD1a other name

A

Von Gierke Disease

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10
Q

GSD2 other name

A

Pompe disease

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11
Q

GSD3 other name

A

Cori disease

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12
Q

GSD4 other name

A

Andersen disease

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13
Q

GSD5 other name

A

McArdle disease

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14
Q

GSD6 other name

A

Hers Disease

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15
Q

GSD7 other name

A

Tarui disease

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16
Q

GSD1 deficiency

A

Glucose-6-phosphatase deficiency

17
Q

GSD2 deficiency

A

Acid maltase (acid a-glucosidase)

18
Q

GSD3 deficiency

A

A-1,6 glucosidase

19
Q

GSD4 deficiency

A

Glucosyl (4:6) transferase

20
Q

GSD5 deficiency

A

muscle glycogen phosphorylase

21
Q

GSD6 deficiency

A

Liver glycogen phosphorylase

22
Q

GSD7 deficiency

A

PFK-1

23
Q

What deficiency presents similarly to Tarui disease?

A

F1,6BP deficiency