Glycogenesis

Question 1

Formation of glycogen

De novo or enlarge

Answer 1

Glycogenesis

Question 2

Mobilizing glycogen

Dietary glycogen breakdown

Answer 2

Glycogenolysis

Question 3

Can represent up to 10% of weight of the liver

1-2% of the weight of skeletal muscle

Answer 3

Glycogen

Question 4

B particle
21 nm in diameter

Consists of up to 55,000 glucose residues

Forms an alpha rosette

Answer 4

Glycogen

Question 5

Branched polymer of glucose

Stored in cytoplasm as either granules (skeletal muscle) or as clusters of granules (liver)

Has central protein core with polyglucosw chains radiating outward to form a sphere

Answer 5

Glycogen

Question 6

Glycogen is stored in the

Answer 6

Cytoplasm

Question 7

Protein in the middle of glycogen

Answer 7

Glycogenin

Question 8

108 gm in well fed state
Glucose reserve
Amount varies with food intake
Stores last 12 to 24 hours

Answer 8

Liver glycogen

Question 9

2x the amount in the liver
Fuel reserve of skeletal muscles only

Absence of glucose 6 phosphatase in muscle

Answer 9

Muscle glycogen

Question 10

Muscle glycogen has no

Answer 10

Glucose 6 Phosphatase

Question 11

Source of glucose during hypoglycemia

Answer 11

Liver glycogen

Question 12

Stored as an energy reserve for muscle contraction

Answer 12

Muscle glycogen

Question 13

White fast twitch muscle fibers employs conversion of glucose to

Answer 13

lactate

Question 14

Red slow twitch muscle fibers employs glucose in this state

Answer 14

oxidized

Question 15

Requirements for glycogen formation

Answer 15

Glycogenin
Glycogen synthase
Glycogen branching enzyme
UDP-glucose pyrophosphorylase

Question 16

Two issues of glycogenesis

Answer 16

Formation of new glycogen particle

Enlargement of existing glycogen particle

Question 17

Precursor of glycogenesis

Glucose donor

Answer 17

UDP glucose

Uridine Diphosphate Glucose

Question 18

Glycogen synthesis is through

Answer 18

Polymerizarion of glucose via

Glycogen synthase
Branching enzyme

Question 19

Core glycogen particle is extended by the

Answer 19

UDP glucose and glycogen synthase

Question 20

Glycogenesis debranching enzyme

Answer 20

Glucosyl alpha-1,4: alpha-1,5 glucosidase

Question 21

Regulatory enzyme of Glycogenesis

Answer 21

Glycogen synthase

Question 22

Synthesis of UDP-Glucose

Formarion of Glycogen Primer

Answer 22

Glycogenesis

Question 23

Serves as a primer for synthesis of new glycogen particles

Answer 23

Glycogenin

Question 24

Breakdown of glycogen to glucose

Answer 24

Glycogenolysis

Question 25

Glycogenolysis is catalyzed by

Answer 25

glycogen phosphorylase

Question 26

Mobilizing glycogen

Answer 26

Glycogenolysis Muscle glycogen will not last for an hour Liver glycogen can be depleted in 12-24 hours

Question 27

To mobilize glycogen, three enzymes are required

Answer 27

Glycogen phosphorylase Debranching enzyme Phosphoglucomutase

Question 28

GlycoGENESIS major enzymes required

Answer 28

Glycogen synthase | Branching enzyme

Question 29

The end product of glycogenolysis is

Answer 29

glucose 6-phosphate

Question 30

Use inorganic phosphate to attack nonreducing ends

Answer 30

Glycogen phosphorylase Phosphorolysis

Question 31

Debranching enzymes responsible for

Answer 31

Breaking Alpha 1,4 bonds | Hydrolyzing Alpha 1,6

Question 32

End product of glycogenolysis

Answer 32

Glucose 6 phosphate

Question 33

Deficiency of Glucose 6 Phosphate Cherub

Answer 33

Von Gierke’s Type I First boyfriend is usually a jerk

Question 34

``` Hypoglycemia Lactic acidosis Hepatomegaly Hyperlipidemia Hyperuricemia Short stature ``` Skin xanthoma Fatty liver

Answer 34

Glucose-6-Phosphatase Deficiency | Von Gierke’s

Question 35

Acid maltase deficiency Alpha 1,4 glucosidase deficiency Accumulation of glycogen in lysosomes

Answer 35

Pompe’s disease

Question 36

Muscle hypotonia | Death from heart failure by age 2

Answer 36

Juvenile onset

Question 37

Muscle distrophy

Answer 37

Adult onset | Pompe’s disease

Question 38

Debranching Enzyme deficiency

Answer 38

Cori’s disease | Type III

Question 39

Fasting hypoglycemia Hepatomegaly Accumulation of characteristic branched polysaccharide Polydextrin Muscle weakness

Answer 39

Type IIIA Debranching Enzyme Deficiency Cori’s Fabre’s

Question 40

Limit dextrinosis As in Type IIIA but no muscle weakness

Answer 40

Type IIIB Debranching Enzyme

Question 41

Absence of enzyme amylo 1:4, 1:6 - transglucosidase Hepatosplenomegaly Accumulation of polysaccharide with few branching points; dearh from heart or liver failure before age 5

Answer 41

Anderson disease ABCD Andersen Branching Cori Debranching

Question 42

Muscle phosphorylase/Myophosphorylase Deficiency Poor exercise tolerance Muscle glycogen abnormally high (2.5 - 4%) Blood lactate very low after exercise

Answer 42

McArdle Disease

Question 43

Hepatic Glycogen Phosphorylase Deficiency

Answer 43

Her’s disease | GSD Type IV

Question 44

Deficiency of Phosphofructokinase

Answer 44

Tarui’s disease | GSD Type IV

Question 45

Poor exercise tolerance Muscle glycogen abnormally high Blood lactate very low after exercise Also hemolytic anemia

Answer 45

Phosphofructokinase deficiency Type IV Tarui’s