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Biochemistry > Glycogenesis > Flashcards

Glycogenesis Flashcards

1
Q

Formation of glycogen

De novo or enlarge

A

Glycogenesis

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2
Q

Mobilizing glycogen

Dietary glycogen breakdown

A

Glycogenolysis

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3
Q

Can represent up to 10% of weight of the liver

1-2% of the weight of skeletal muscle

A

Glycogen

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4
Q

B particle
21 nm in diameter

Consists of up to 55,000 glucose residues

Forms an alpha rosette

A

Glycogen

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5
Q

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

A

Glycogen

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6
Q

Glycogen is stored in the

A

Cytoplasm

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7
Q

Protein in the middle of glycogen

A

Glycogenin

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8
Q

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

A

Liver glycogen

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9
Q

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

Absence of glucose 6 phosphatase in muscle

A

Muscle glycogen

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10
Q

Muscle glycogen has no

A

Glucose 6 Phosphatase

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11
Q

Source of glucose during hypoglycemia

A

Liver glycogen

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12
Q

Stored as an energy reserve for muscle contraction

A

Muscle glycogen

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13
Q

White fast twitch muscle fibers employs conversion of glucose to

A

lactate

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14
Q

Red slow twitch muscle fibers employs glucose in this state

A

oxidized

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15
Q

Requirements for glycogen formation

A

Glycogenin
Glycogen synthase
Glycogen branching enzyme
UDP-glucose pyrophosphorylase

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16
Q

Two issues of glycogenesis

A

Formation of new glycogen particle

Enlargement of existing glycogen particle

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17
Q

Precursor of glycogenesis

Glucose donor

A

UDP glucose

Uridine Diphosphate Glucose

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18
Q

Glycogen synthesis is through

A

Polymerizarion of glucose via

Glycogen synthase
Branching enzyme

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19
Q

Core glycogen particle is extended by the

A

UDP glucose and glycogen synthase

20
Q

Glycogenesis debranching enzyme

A

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

21
Q

Regulatory enzyme of Glycogenesis

A

Glycogen synthase

22
Q

Synthesis of UDP-Glucose

Formarion of Glycogen Primer

A

Glycogenesis

23
Q

Serves as a primer for synthesis of new glycogen particles

A

Glycogenin

24
Q

Breakdown of glycogen to glucose

A

Glycogenolysis

25
Q

Glycogenolysis is catalyzed by

A

glycogen phosphorylase

26
Q

Mobilizing glycogen

A

Glycogenolysis

Muscle glycogen will not last for an hour

Liver glycogen can be depleted in 12-24 hours

27
Q

To mobilize glycogen, three enzymes are required

A

Glycogen phosphorylase
Debranching enzyme
Phosphoglucomutase

28
Q

GlycoGENESIS major enzymes required

A

Glycogen synthase

Branching enzyme

29
Q

The end product of glycogenolysis is

A

glucose 6-phosphate

30
Q

Use inorganic phosphate to attack nonreducing ends

A

Glycogen phosphorylase

Phosphorolysis

31
Q

Debranching enzymes responsible for

A

Breaking Alpha 1,4 bonds

Hydrolyzing Alpha 1,6

32
Q

End product of glycogenolysis

A

Glucose 6 phosphate

33
Q

Deficiency of Glucose 6 Phosphate

Cherub

A

Von Gierke’s
Type I

First boyfriend is usually a jerk

34
Q 
Hypoglycemia
Lactic acidosis
Hepatomegaly
Hyperlipidemia
Hyperuricemia
Short stature

Skin xanthoma

Fatty liver

A

Glucose-6-Phosphatase Deficiency

Von Gierke’s

35
Q

Acid maltase deficiency
Alpha 1,4 glucosidase deficiency

Accumulation of glycogen in lysosomes

A

Pompe’s disease

36
Q

Muscle hypotonia

Death from heart failure by age 2

A

Juvenile onset

37
Q

Muscle distrophy

A

Adult onset

Pompe’s disease

38
Q

Debranching Enzyme deficiency

A

Cori’s disease

Type III

39
Q

Fasting hypoglycemia
Hepatomegaly
Accumulation of characteristic branched polysaccharide
Polydextrin

Muscle weakness

A

Type IIIA Debranching Enzyme Deficiency
Cori’s
Fabre’s

40
Q

Limit dextrinosis

As in Type IIIA but no muscle weakness

A

Type IIIB Debranching Enzyme

41
Q

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

A

Anderson disease

ABCD
Andersen Branching
Cori Debranching

42
Q

Muscle phosphorylase/Myophosphorylase Deficiency

Poor exercise tolerance

Muscle glycogen abnormally high (2.5 - 4%)
Blood lactate very low after exercise

A

McArdle Disease

43
Q

Hepatic Glycogen Phosphorylase Deficiency

A

Her’s disease

GSD Type IV

44
Q

Deficiency of Phosphofructokinase

A

Tarui’s disease

GSD Type IV

45
Q

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

Also hemolytic anemia

A

Phosphofructokinase deficiency
Type IV
Tarui’s

Biochemistry (32 decks)

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