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Biology and Biochem > Glycogen, Gluconeogenesis, and HMP shunt > Flashcards

Glycogen, Gluconeogenesis, and HMP shunt Flashcards

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1
Q

Hers disease deficient in

A

Hepatic glycogen phosphorylase

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2
Q

What is the rate limiting enzyme in glycogenolysis

A

Glycogen phosphorylase

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3
Q

Purpose of Malate shuttle in Gluconeogenesis

A

Transports OAA out of mitochondria into cytoplasm

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4
Q

Von Gierke clinical

A
  1. Severe hypoglycemia
  2. Lactic acidosis
  3. Hepatomegaly
  4. hyperlipidemia
  5. Hyperuricemia
  6. Short, doll-like face
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5
Q

Glycogen phosphorylase is stimulated by

A

Glucagon

Epinepherine

AMP

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6
Q

PEP carboxykinase converts

A

OAA to PEP

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7
Q

Cori disease deficient in

A

Glycogen debranching enzyme

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8
Q

McArdle disease deficient in

A

Muscle glycogen phosphorylase

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9
Q

Glucose-6-phosphatase converts

A

Glucose 6-P to Glucose

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10
Q

Andersen (amylopectinosis) disease deficient in

A

Branching enzyme

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11
Q

G6PD genetics

A

x-linked recessive

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12
Q

Glucose 6-phosphatase converts

A

Glucose 6-P to Glucose

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13
Q

Andersen clinical

A

Infantile hypotonia

Cirrhosis

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14
Q

What are the 2 major functions of HMP shunt

A

NADPH production

Source of ribose 5-phosphate for nucleotide synthesis

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15
Q

Pyruvate Carboxylase converts

A

Pyruvate to OAA

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16
Q

McArdle clinical

A

Muscle cramps

Weakness on exercise

Myoglobinuria

17
Q

High NADH due to alcohol favors formation of

A

Lactate from Pyruvate

Malate from OAA

Glycerol 3-phosphate from DHAP

18
Q

Glycogen synthase converts

A

UDP-Glucose to Glycogen

19
Q

Pyruvate carboxylase coenzyme

A

Biotin

20
Q

Cori clinical

A

Mild Hypoglycemia

Liver enlargement

21
Q

Pompe clinical

A

Cardiomegaly

Muscle weakness

22
Q

Glycogen phosphorylase converts

A

Glycogen to Glucose 1-P

23
Q

Pompe disease deficient in

A

Lysosomal α1,4-glucosidase

24
Q

Fructose-1,6-bisphosphatase converts

A

Fructose 1,6-BP to Fructose 6-P

25
Q

G6PD deficiency results in

A

Hemolytic anemia

Symptoms resembling CGD

Heinz bodies

26
Q

Von Gierke disease deficient in

A

Glucose-6-phosphatase

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