Glycogen

Question 1

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

Answer 1

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

Question 2

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

Answer 2

Glycine, arginine, and methionine

Question 3

___ acts as high energy reservoir in the muscle

Answer 3

Creatine phosphate

Question 4

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

Answer 4

Creatine phosphate

Question 5

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

Answer 5

Creatine kinase known as CK-MB in the plasma

Question 6

Creatine and creatine phosphate form ___ when degraded

Answer 6

Creatinine

Question 7

Creatinine is a normal constituent of ___

Answer 7

Urine

Question 8

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

Answer 8

Proportional

Question 9

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

Answer 9

Decreases

Question 10

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

Answer 10

Kidney disease

Question 11

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

Answer 11

Increase

Question 12

Where are the main stores of glycogen found?

Answer 12

Liver and Muscle

Question 13

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

Answer 13

~ 100g (10% of fresh weight)

Question 14

How many grams of glycogen does resting muscle contain?

Answer 14

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

Question 15

When is muscle glycogen extensively degraded?

Answer 15

During strenuous exercise

Question 16

When is liver glycogen degraded?

Answer 16

For the maintenance of blood glucose level

Question 17

The synthesis of glycogen is called

Answer 17

Glycogenesis

Question 18

The catabolism of glycogen is called

Answer 18

Glycogenolysis

Question 19

What are the major enzymes in glycogenesis?

Answer 19

Glycogen synthase and branching enzyme

Question 20

Where is the subcelluar site of glycogenesis?

Answer 20

Cytosol

Question 21

What energy forms are required for glycogenesis?

Answer 21

ATP and UTP

Question 22

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

Answer 22

Phosphoglucomutase

Question 23

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

Answer 23

UDPG pyrophosphorylase

Question 24

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

Answer 24

Glycogen synthase

Question 25

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

Answer 25

Branching Enzyme

Question 26

What serves as a primer in Glycogenesis?

Answer 26

Glycogenin

Question 27

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

Answer 27

UDPG

Question 28

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

Answer 28

Glycogen sythase

Question 29

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

Answer 29

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

Question 30

What are the major enzymes of glycogenolysis?

Answer 30

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

Question 31

What is the sub cellular site of glycogenolysis?

Answer 31

Cytosol

Question 32

What are the end products of glycogenolysis?

Answer 32

Liver: glucose Muscle: glucose-6-phophate

Question 33

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

Answer 33

Glucose-1-phosphate

Question 34

Glycogen phosphorylase is ___ dependent

Answer 34

PLP dependent

Question 35

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

Answer 35

PLP (Pyridoxal phosphate)

Question 36

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

Answer 36

Glycogen phosphorylase

Question 37

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

Answer 37

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

Question 38

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

Answer 38

Alpha-glucosidase

Question 39

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

Answer 39

Glycogenolysis; glycogenesis

Question 40

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

Answer 40

Glycogenesis; glycogenolysis

Question 41

What are the main regulatory enzymes for glycogen metabolism?

Answer 41

Glycogen synthase and glycogen phosphorylase

Question 42

At what two levels is glycogen metabolism regulated?

Answer 42

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

Question 43

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

Answer 43

Glucagon

Question 44

___ exert their action on both the liver and the muscles

Answer 44

Insulin and Epinephrine

Question 45

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

Answer 45

Von Gierke Disease Deficiency of glucose-6-phosphatase in liver

Question 46

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

Answer 46

No name Deficiency of glucose-6-phosphate translocate in ER

Question 47

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

Answer 47

Pompe disease Deficiency of lysosomal alpha-glucosidase (acid maltase)

Question 48

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

Answer 48

Cori disease Deficiency of liver and muscle deb ranching enzyme

Question 49

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

Answer 49

Andersen disease/ amylopectinosis Deficiency of branching enzyme

Question 50

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

Answer 50

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

Question 51

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

Answer 51

Hers' disease Deficiency of glycogen phosphorylase in the liver