Glycogen Flashcards

1
Q

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

A

In the glycogen chains

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2
Q

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

A

At the branch points of glycogen

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3
Q

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

A

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

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4
Q

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

A

Glucose-6-phosphatase

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5
Q

What is the main role of hepatic glycogen?

A

Used to generate glucose in times of hypoglycemia

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6
Q

What is the main role of muscle glycogen?

A

Used to generate ATP during exercise

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7
Q

What is the function of glycogen synthase?

A

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

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8
Q

What is the function of branching enzyme?

A

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

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9
Q

What are the enzymes of glycogen degradation?

A

Glycogen Phosphorylase and Debranching Enzyme

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10
Q

What are some of the secondary messenger systems for glycogen?

A

cAMP
PI
Calcium

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11
Q

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

A

Activates glycogen degradation and inhibits glycogen synthesis

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12
Q

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

A

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

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13
Q

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

A

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

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14
Q

What is the effect of glycogen debranching enzyme deficiency?

A

Increased glycogen in the muscle and liver but is mild

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15
Q

What is the effect of glycogen branching enzyme deficiency?

A

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

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16
Q

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

A

Moderate amount of glycogen increase with limited ability to exercise

17
Q

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

A

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

18
Q

What is the effect of PFK-1 deficiency?

A

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

19
Q

What is the effect of phosphorylase b kinase deficiency?

A

Increased amount in liver with mild liver enlargement and hypoglycemia

20
Q

What is generated from the pentose phosphate pathway?

A

NADPH

Ribose-5-Phosphate

21
Q

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

A

Glucose-6-Phosphate Dehydrogenase

22
Q

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

A

Hemolytic anemia