Glycogen Flashcards

1
Q

What are the ways to generate ATP in skeletal muscle during exercise?

A
  1. Aerobic Cellular Respiration
  2. Anaerobic Glycolysis
  3. Direst phosphorylation of ADP by creatine kinase
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2
Q

What 3 amino acids are needed for the synthesis of creatine?

A

Glycine, arginine, and methionine

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3
Q

___ acts as high energy reservoir in the muscle

A

Creatine phosphate

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4
Q

___ can be rapidly mobilized to form ATP during the first few moments of intense muscular contraction

A

Creatine phosphate

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5
Q

The presence of this isoenzyme is indicative of heart damage and is used in the diagnosis of myocardial infarction.

A

Creatine kinase known as CK-MB in the plasma

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6
Q

Creatine and creatine phosphate form ___ when degraded

A

Creatinine

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7
Q

Creatinine is a normal constituent of ___

A

Urine

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8
Q

The amount of creatinine excreted in the urine is ___ to the total creatine phosphate in the body, therefore to the muscle mass

A

Proportional

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9
Q

If the muscle mass decreases, such as in paralysis or muscle dystrophy, the creatinine content in the urine ___

A

Decreases

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10
Q

Determination of creatinine level in plasma is an important investigation in ___

A

Kidney disease

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11
Q

___ of plasma creatinine is a notable feature seen in renal failure

A

Increase

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12
Q

Where are the main stores of glycogen found?

A

Liver and Muscle

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13
Q

How many grams of glycogen does well-fed adult liver contain?

A

~ 100g (10% of fresh weight)

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14
Q

How many grams of glycogen does resting muscle contain?

A

~ 400g (1-2% of fresh weight)

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15
Q

When is muscle glycogen extensively degraded?

A

During strenuous exercise

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16
Q

When is liver glycogen degraded?

A

For the maintenance of blood glucose level

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17
Q

The synthesis of glycogen is called

A

Glycogenesis

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18
Q

The catabolism of glycogen is called

A

Glycogenolysis

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19
Q

What are the major enzymes in glycogenesis?

A

Glycogen synthase and branching enzyme

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20
Q

Where is the subcelluar site of glycogenesis?

A

Cytosol

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21
Q

What energy forms are required for glycogenesis?

A

ATP and UTP

22
Q

What transforms Glucose-6-phophate to Glucose-1-phosphate?

A

Phosphoglucomutase

23
Q

What transforms Glucose-1-phosphate to UDP Glucose (UDPG)?

A

UDPG pyrophosphorylase

24
Q

What transforms UDP Glucose to Glycogen (with 1->4 glucosyl units)?

A

Glycogen synthase

25
Q

What transforms Glucose with 1->4 branching to Glucose with 1->4 and 1->6 glucosyl units?

A

Branching Enzyme

26
Q

What serves as a primer in Glycogenesis?

A

Glycogenin

27
Q

What acts as a carrier of the glucose for the synthesis of glucose?

A

UDPG

28
Q

What incorporates glucose units to the glycogen primes by forming alpha 1->4 glycosidic bonds?

A

Glycogen sythase

29
Q

Where does branching enzyme from glucose residues to attach it to another glucosyl residue? How many are removed?

A

Removed from the nonreducing end; a set of 6-8

30
Q

What are the major enzymes of glycogenolysis?

A

Glycogen phosphorylase and debranching enzyme (with 4:4 transferase and 1:6 glucosidase activity)

31
Q

What is the sub cellular site of glycogenolysis?

A

Cytosol

32
Q

What are the end products of glycogenolysis?

A

Liver: glucose
Muscle: glucose-6-phophate

33
Q

What is the major product formed as a result of glycogenolysis?

A

Glucose-1-phosphate

34
Q

Glycogen phosphorylase is ___ dependent

A

PLP dependent

35
Q

What is the coenzyme form of B-complex vitamin, Pyridoxine (B6)?

A

PLP (Pyridoxal phosphate)

36
Q

This enzyme sequentially removes the glycosyl residues from the non-reducing ends as glucose-1-phosphate by phosphorolysis?

A

Glycogen phosphorylase

37
Q

A small amount of glycogen is continuously degraded by the lysosomal enzyme ___

A

alpha-glucosidase (acid maltase/ alpha 1,4-glucosidase)

38
Q

Deficiency of this enzyme causes accumulation of glycogen in lysosomes (Pompe disease or type II glycogen storage disease)

A

Alpha-glucosidase

39
Q

In skeletal muscle, ___ occurs during active exercise, ___ begins as soon as the muscle is at rest

A

Glycogenolysis; glycogenesis

40
Q

In the liver, ___ accelerates in the well-fed state, ___ increases in the fasting state

A

Glycogenesis; glycogenolysis

41
Q

What are the main regulatory enzymes for glycogen metabolism?

A

Glycogen synthase and glycogen phosphorylase

42
Q

At what two levels is glycogen metabolism regulated?

A

Hormonal (by phosphorylation/dephosphorylation): to meet the needs of the whole body

Allosteric (by effector molecules): regulation to meet the needs of a particular tissue

43
Q

___ regulates glycogen metabolism in the liver, but not in the muscles?

A

Glucagon

44
Q

___ exert their action on both the liver and the muscles

A

Insulin and Epinephrine

45
Q

What is type 1a glycogen storage disease? What is it’s deficiency?

A

Von Gierke Disease

Deficiency of glucose-6-phosphatase in liver

46
Q

What is type 1b glycogen storage disease? What is it’s deficiency?

A

No name

Deficiency of glucose-6-phosphate translocate in ER

47
Q

What is type II glycogen storage disease? What is it’s deficiency?

A

Pompe disease

Deficiency of lysosomal alpha-glucosidase (acid maltase)

48
Q

What is type III glycogen storage disease? What is it’s deficiency?

A

Cori disease

Deficiency of liver and muscle deb ranching enzyme

49
Q

What is type IV glycogen storage disease? What is it’s deficiency?

A

Andersen disease/ amylopectinosis

Deficiency of branching enzyme

50
Q

What is type V glycogen storage disease? What is it’s deficiency?

A

McArdle disease

Deficiency of muscle glycogen phosphorylase (myophosphorylase); liver enzyme is normal

51
Q

What is type VI glycogen storage disease? What is it’s deficiency?

A

Hers’ disease

Deficiency of glycogen phosphorylase in the liver