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Flashcards in Glycogen Deck (22)
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1

Where are alpha-1,4 linkages in glycogen found?

In the glycogen chains

2

Where are alpha-1,6 linkages in glycogen found?

At the branch points of glycogen

3

Why is glucose stored in the form of a branched molecule like glycogen?

Branches allow for more efficient degradation than if it were a single long chain

4

What enzyme is present in the liver that is not in the muscles and is important in glycogenolysis and gluconeogenesis?

Glucose-6-phosphatase

5

What is the main role of hepatic glycogen?

Used to generate glucose in times of hypoglycemia

6

What is the main role of muscle glycogen?

Used to generate ATP during exercise

7

What is the function of glycogen synthase?

Adds glucose units in an alpha-1,4-linkage.

8

What is the function of branching enzyme?

Adds glucose units in an alpha-1,6-linkage.

9

What are the enzymes of glycogen degradation?

Glycogen Phosphorylase and Debranching Enzyme

10

What are some of the secondary messenger systems for glycogen?

cAMP
PI
Calcium

11

What is the effect of the secondary messenger systems on glycogen?

Activates glycogen degradation and inhibits glycogen synthesis

12

What is the effect of Glucose-6-Phosphatase deficiency?

Increased glycogen in the liver and kidney that can lead to hypoglycemia and enlarged liver.

13

What is the effect of 1,4 alpha glucosidase deficiency?

Massive increase in glycogen in all organs and death before age 2 normally

14

What is the effect of glycogen debranching enzyme deficiency?

Increased glycogen in the muscle and liver but is mild

15

What is the effect of glycogen branching enzyme deficiency?

Normal amount of glycogen but they are found in long branches and cause liver cirrhosis that leads to death before age 2

16

What is the effect of glycogen phosphorylase deficiency in the muscle?

Moderate amount of glycogen increase with limited ability to exercise

17

What is the effect of glycogen phosphorylase deficiency in the liver?

Increased amount of glycogen and is similar to a G6Pi deficiency but is milder.

18

What is the effect of PFK-1 deficiency?

Increased glycogen in the muscle and is similar to glycogen phosphorylase deficiency

19

What is the effect of phosphorylase b kinase deficiency?

Increased amount in liver with mild liver enlargement and hypoglycemia

20

What is generated from the pentose phosphate pathway?

NADPH
Ribose-5-Phosphate

21

What is the rate limiting enzyme of the pentose phosphate pathway?

Glucose-6-Phosphate Dehydrogenase

22

What is deficiency of Glucose-6-Phosphate Dehydrogenase associated with?

Hemolytic anemia

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