Glycogen

Question 1

where do you find glycogen?

Answer 1

• liver

- muscle

Question 2

starving hypoglycemia, elevated glycogen

Answer 2

GLUT2 deficiency

Question 3

short-term glucose buffer

Answer 3

glycogen

Question 4

High glucose would _____ proteins, pose a major _____ problem and would leak out due to _______ transport

Answer 4

High glucose would glycate proteins, pose a major osmotic problem and would leak out due to facilitated transport

Question 5

First step of synthesis of glycogen

Answer 5

G6P—-> G1P by phsphoglucomutase

Question 6

G1P needs to be activated.. how?

Answer 6

using UDP-glucose pyrophosphorylase to make UDP-Glucose

Question 7

polymer synthesis involves UDP glucose and turning into

Answer 7

Glycogen alpha 1-4 by glycogen synthase

Question 8

Glycogen alpha 1-4 can add

Answer 8

glycgoen alpha 1-6 via a branching enzyme

Question 9

Glycogen synthase is limiting and a regulated step… why?

Answer 9

elongates by adding one glucose at a time starting with UDP-Glucose primer

Question 10

Breakdown of glycogen involves glycogen phosphorylase which and the product is

Answer 10

cleaves one glucose at a time and the product is G1P

Question 11

transfers three residues of a branching chain to a different chain

Answer 11

debranching enzyme

Question 12

debranching enzyme cleaves the remaining glucose by ______ resulting in

Answer 12

debranching enzyme cleaves the remaining glucose by hydrolysis resulting in free glucose

Question 13

muscle does not have this enzyme that is used in the breakdown of glycogen

Answer 13

glucose 6 phosphatase

Question 14

maintain blood glucose concentration

Answer 14

insulin and glucagon

Question 15

releases fuel when needed for stress response: via cyclic AMP and protein kinase A

Answer 15

epinephrine

Question 16

chronically stimulates glycogenolysis

Answer 16

cortisol

Question 17

release of fuel supply within muscle: via calcium release, calmodulin and protein kinase A

Answer 17

nerve-muscle stimulus

Question 18

high glucose, G6P and G1) favors synthesis

Answer 18

dietary intake

Question 19

inhibits glycogen breakdown

Answer 19

F1P

Question 20

increased glycogen content slows synthesis

Answer 20

autoregulation

Question 21

Gycogen phosphorylase is ________than P-Glycogen phosphorylase a

Answer 21

less active

Question 22

Glycogen synthase is _____ than P-glycogen synthase

Answer 22

more active

Question 23

P-glycogen phosphorylase a is induced by

Answer 23

glucagon

Question 24

Glycogen synthase is induced by

Answer 24

insulin

Question 25

in liver ______ stimulates glycogen breakdown

Answer 25

epinephrine

Question 26

______ activates phosphorylase kinase

Answer 26

calcium

Question 27

in muscle, ______ or _______ stimulates glycogen breakdown

Answer 27

epinephrine or nerve stimulation

Question 28

In a glucose tolerance test, a patient ingests a large amount of glucose. What will happen in a normal patient? A. Inhibition of glycogen synthetase phosphatase B. Increase rate of lactate formation by erythrocytes C. Enhanced phosphorylase activity in the pancreas D. Increased ratio of phosphorylase a to phosphorylase b E. Enhanced glycogen synthase activity in liver

Answer 28

E. Enhanced glycogen synthase activity in liver

Question 29

Glucose-6-phosphatase defect

Answer 29

von Gierke disease

Question 30

von Gierke disease type 1B

Answer 30

transported defect

Question 31

Lysosomal alpha 1-4 glucosidase defect

Answer 31

Pompe disease

Question 32

Debranching enzyme defect

Answer 32

Cori's disease

Question 33

Branching enzyme defect

Answer 33

Andersen's disease

Question 34

A defect in which of the following enzymes would lead to glycogen with a defective structure (as opposed to a change in amount). ``` A. adenyl cyclase B. glycogen branching enzyme C. glycogen synthase D. phosphorylase kinase E. phosphorylase. ```

Answer 34

B. glycogen branching enzyme

Question 35

A baby presents with growth retardation, hepatomegaly and hypoglycemia. Liver biopsy shows small dextrin-like molecules in the cytoplasm of cells. What is likely to be wrong? ``` A. Type I GSD von Gierke B. Type II GSD Pompe C. Type III GSD Cori disease D. Type IV GSD Andersen E. Type V GSD McArdle ```

Answer 35

C. Type III GSD Cori | disease

Question 36

Skeletal muscle glycogen phosphorylase defect

Answer 36

McArdle disease

Question 37

Liver glycogen Phosphorylase defect

Answer 37

Hers disease

Question 38

``` A college girl taking up organized sports for the first time complains of severe leg cramps after trying out for the soccer team. She also noticed a reddish tinge in her urine that night. You order an ischemic forearm exercise test that shows no lactate. Where is the defect? A. Glucose 6-phosphatase B. Acid maltase C. alpha 1-6 glucosidase D. Glycogen phosphorylase E. Cystathionine synthetase ```

Answer 38

D. Glycogen phosphorylase

Question 39

phosphofructokinase defect

Answer 39

Tauri's disease

Question 40

excess glucose stored as

Answer 40

glycogen

Question 41

Glucose is converted to ______ then into ______.

Answer 41

Glucose is converted to G6P then into G1P

Question 42

Where is glycogen found?

Answer 42

liver and muscles

Question 43

there is a greater amount of glycogen in _____... why?

Answer 43

muscles because there are more muscles than liver

Question 44

why make glycogen? (4)

Answer 44

1. glucose can leak out 2. osmotic pressure problem 3. glycation causes tissue damage- glucose causes it 4. easier regulation

Question 45

enzyme that converts G6P to G1P

Answer 45

phosphoglucomutase

Question 46

which enzyme adds UTP to GIP?

Answer 46

UDP- Glucose pyrophsphorylase activates glucose

Question 47

which is the limiting/regulating step in glycogen synthesis?

Answer 47

polymer synthesis- UDP-glucose to glycogen

Question 48

glycogen synthase requires a

Answer 48

primer- usually an existing glycogen strand

Question 49

if no glycogen exists, then the enzyme ______ will act as a primer adding UDP-Glucose on _____

Answer 49

if no glycogen exists, then the enzyme Glycogenin will act as a primer adding UDP-Glucose on Tyr 194

Question 50

branching allows for

Answer 50

faster mobilization of glycogen

Question 51

enzyme that breaks down glycogen

Answer 51

glycogen phosphorylase

Question 52

Glycogen phosporylase is the ________ step and will use ____ to cleave glycogen and create G1P as a product.

Answer 52

Glycogen phosporylase is the limiting step and will use Pi to cleave glycogen and create G1P as a product.

Question 53

debranching enzyme has both ____ and _____ activity

Answer 53

transferase and hydrolase

Question 54

strongest promoter of glycolysis

Answer 54

F26BP

Question 55

muscle glycogen breakdone results in _____ but not in the liver

Answer 55

glycolysis

Question 56

Glycogen phosphorylase is activated by

Answer 56

AMP and calcium

Question 57

Glycogen phosphorylase is inhibited by

Answer 57

ATP and G6P

Question 58

phosphorylase a is in the

Answer 58

active form

Question 59

phosphorylase b is in the

Answer 59

inactive form

Question 60

______ will cause the formation of cAMP which will activate phosphorylase kinase leading to ____

Answer 60

Glucagon leads to glycogen breakdown

Question 61

Insulin will _____________ glycogen phosphorylase leading to its _______ and activating

Answer 61

Insulin will dephosphorylate leading to its inactivation and activating glycogen synthesis

Question 62

epinephrine leads to

Answer 62

glycogen breakdown

Question 63

pompe disease leads to

Answer 63

cardiomegaly

Question 64

fatal due to the altered structure of glycogen

Answer 64

Anderson's

Question 65

red tinge urine

Answer 65

mcArdle

Question 66

normal glycogen structure but altered amount

Answer 66

Hers disease

Question 67

fructose helps

Answer 67

Tauri's disease