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Biochemistry > Glycogen, Galactose, Fructose, Polyol, Uronic, PPPMetabolism > Flashcards

Glycogen, Galactose, Fructose, Polyol, Uronic, PPPMetabolism Flashcards

1
Q

What is the major storage form of glucose in animals?

A

glycogen

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2
Q

How many grams of glycogen is stored in the muscle and liver?

A

500g

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3
Q

The primary glycosidic bonds is located at

A

alpha (1,4)

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4
Q

Branch points of glycogen happens after how many residues?

A

8 to 10 residues

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5
Q

The branch points of glycogen happens where

A

alpha(1,6)

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6
Q

Glycogenesis happens where

A
  1. liver and muscle in the cytosol
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7
Q

What is the substrate of glycogenesis?

A

alpha-D-glucose

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8
Q

what enzyme is the rate-limiting of glycogenesis?

A

glycogen synthase

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9
Q

What protein serves as the primer for glycogen synthesis when glycogen is completely depleted?

A

glycogenin

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10
Q

What are the enzymes needed to synthesize UDP-Glucose

A
  1. Phosphoglucomutase

2. UDP-glucose pyrophosphorylase

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11
Q

Cite the steps in synthesizing UDP-glucose

A
  1. G6P to G1P

2. G1P to UDP-glucose

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12
Q

What enzyme is needed for glycogen chain elongation?

A

glycogen synthase

the rate limiting step

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13
Q

What is the glycogen branching enzyme

A

Amylo alpha (1–>4) to alpha (1,6) transglucosidase

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14
Q

What is the substrate for glycolysis

A

Glycogen

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15
Q

What are the products of glycogenolysis?

A
  1. Glucose in liver

2. Glucose 6 phosphate in muscle

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16
Q

What is the rate limiting step of glycogenolysis?

A

glycogen phosphorylase

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17
Q

What enzyme cleave the alpha 1,4 bonds?

A

glycogen phosphorylase

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18
Q

What is the coenzyme of glycogen phosphorylase?

A

pyridoxal phosphate

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19
Q

When will glycogen phosphorylase stops its action?

A

if only 4 glucosyl units remains (called limit dextrin)

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20
Q

What are the debranching enzyme of glycogen?

A
  1. alpha 1,4 glucantransferase

2. amylo alpha(1,6) glucosidase

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21
Q

What cleaves the 3 glucose units in the limit dextrin?

A

alpha 1,4 glucaltransferase

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22
Q

What cleaves the branching glucose molecule in glycogen?

A

amylo alpha 1,6 glucosidase

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23
Q

What enzyme converts glucose-1-P to Glucose-6P in the muscle or to glucose in the liver

A

phosphoglucomutase

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24
Q

Around 1-3% of glycogen is degraded via this enzyme

A

alpha 1,4 glucosidase or acid maltase

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25
Q

[Phosphorylation/ Dephosphorylation]

glycogen synthase when active

A

dephosphorylated

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26
Q

[Phosphorylation/ Dephosphorylation]

glycogen phosphorylase when active

A

phosphorylated

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27
Q

[Lysosomal Storage disease]

glucose 6 phosphatase deficiency

A

Von Gierke

Ia

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28
Q

[Lysosomal Storage disease]

lysosomal alpha glucosidase

A

Pompe

II

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29
Q

[Lysosomal Storage disease]

debranching enzyme

A

Cori

III

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30
Q

[Lysosomal Storage disease]

branching enzyme

A

Andersen

IV

31
Q

[Lysosomal Storage disease]

muscle phosphorylase

A

McArdle

V

32
Q

[Lysosomal Storage disease]

liver phosphorylase

A

Hers disease

VI

33
Q

[Lysosomal Storage disease]

Increased glycogen in liver and renal tubule,

hypoglycemia

lactic acidosis, ketosis

hyperlipemia

A

Von Gierke

34
Q

[Lysosomal Storage disease]

increase in glycogen lysosome
hypotonia, death from heart failure by age 2

A

Pompe

35
Q

[Lysosomal Storage disease]

fasting hypoglycemia
hepatomegaly in infancy
increase in limit dextrin
muscle weakness

A

Cori disease

debranching enzyme

36
Q

[Lysosomal Storage disease]

hepatosplenomegaly
increasepolysaccharide with few branch points,
death from heart failure before age 5

A

Andersen

branching enzyme

37
Q

[Lysosomal Storage disease]

poor exercise tolerance, muscle cramps, and myoglobinuria but no lactic acidosis, increase muscle glycogen

A

Mcardle disease

38
Q

[Lysosomal Storage disease]

hepatomegaly, mild hypoglycemia

A

Hers disease

39
Q

[Galactose Metabolism]

enzyme needed to convert Galactose to Galactose 1 phosphate

A

Galactokinase

40
Q

[Galactose Metabolism]

enzyme needed to convert Galactose1P to UDP galactose

A

UDP-hexose 4 epimerase

41
Q

[Diagnose]

galactosemia + galactusuria
cataracts in early childhood

A

galaktokinase

42
Q

[Diagnose]

galactosemia, galactosuria, cataracts, jaundice, vomiting, diarrhea

poor growth, severe mental retardation, liver damage

premature ovarial failure

A

Classic Galactosemia

Galactose1-phosphate uridyl transferase deficiency

43
Q

[Fructose Metabolism]

What is the enzyme needed to convert Fructose to Fructose-1-P?

A

Fructokinase or Hexokinase

44
Q

[Fructose Metabolism]

What is the enzyme needed to convert Fructose1P to DHAP + glyceraldehyde?

A

Aldolase B

45
Q

[Fructose Metabolism]

Aldolase B is found in what body organ?

A

Liver

46
Q

[Fructose Metabolism]

Aldolase C is found in what organ?

A

brain

47
Q

[Fructose Metabolism]

What type of aldolase that is present in most tissues?

A

Aldosalse A

48
Q

[Fructose Metabolism]

Enzyme involved in asymptomatic patients but with fructosuria

A

Fructokinase deficiency

49
Q

[Diagnosis]

profound hypoglycemia, vomiting after consumption of fructose,

jaundice, hemorrhage, hepatomegaly, liver failure, renal dysfunction, hyperuricemia, lactic acidosis, death

symptoms appear after weaning from milk

A

aldolase B deficiency

50
Q

What pathway is used when glucose is converted to sorbitol

A

polyol pathway

Enzyme: Aldose reductase

51
Q

[Polyol pathway]

What is the enzyme essential to convert glucose to sorbitol?

A

Aldose reductase

needs NADPH + H+

52
Q

[Polyol pathway]

What enzyme is needed to convert sorbitol to fructose

A

Sorbitol dehydrogenase

53
Q

what part of the body has no sorbitol dehydrogenase?

A
  1. Retina
  2. Lens
  3. Kidney
  4. Schwann cells
54
Q

Sorbitol dehydrogenase are found in what organs?

A
  1. Liver
  2. Ovaries
  3. Seminal vesicles
55
Q

[Diagnosis]

adult african or asian descent

flatulence and diarrhea after ingestion of dairy products

A

Lactose intolerance

Lactase deficiency

56
Q

[Diagnosis]

intolerance after ingestion of sugar
Inuit people of greenland and canada

A

sucrase isomaltase complex deficiency

57
Q

What pathway is an alternative pathway for oxidation of glucose in the liver?

A

uronic acid pathway

58
Q

What is the main pathway for production of glucoronic and iduronic acid

A

uronic acid pathway

59
Q

What glucose derivative is an essential component of glycosaminoglycans?

A

glucoronic acid

60
Q

Glucoronic acid is required in detoxification reactions of this compounds

A
  1. Bilirubin
  2. Steroids
  3. Morphine and other drugs
61
Q

What enzyme is implicated in primates’ inability to synthesize vitamin C

A

L-gulunolactose oxidase

62
Q

[Uronic pathway]

What converts UDP glucose to UDP glucoronic acid?

A

UDP Glucose dehydrogenase

63
Q

[Diagnose]

increased xylulose in the urine; benign with no clinical consequence

A

Xylulose reductase deficiency

64
Q

What is the main pathway produces NADPH?

A

Pentose Phosphate Pathway

65
Q

Ribose 5-phosphate is produced in what pathway

A

Hexose Monophosphate Shunt (Pentose Phosphate Pathway)

to use 5-carbon sugar

66
Q

[Pentose Phosphat Pathway]

What is its substrate?

A

Glucose-6-phosphate

67
Q

[Pentose Phosphat Pathway]

What is the rate limiting step

A

Glucose6Phosphate to 6phosphogluconate

via Glucose 6 phosphate dehydrogenase

68
Q

[Pentose Phosphat Pathway]

What are the products of the oxidative, irreversible phase

A

Enzyme: G6P dehydrogenase

pdt: 2NADPH +R5P

69
Q

[Pentose Phosphat Pathway]

what are the products of the second, reversible phase?

A

Enzyme: transketolase, Transaldolase

Pdts:

  1. R5P
  2. F6P
  3. G3P
  4. Other CHO
70
Q

[Pentose Phosphat Pathway]

what is the cofactor of transketolase?

A

Thiamine

71
Q

[Pentose Phosphate Pathway: Clinical correlate]

Glutathione peroxidase removes what cellular byproduct?

A

H2O2

72
Q

What enzyme is the most common disease producing enzyme abnormality in humans?

A

G6PD deficiency

73
Q

What is the role of gluthathione reductase?

Cite the reaction involved

A
  1. NADPH + NADP

2. GSSG to 2G-SH (FAD needed)

74
Q

What is the role of glutathione peroxidase?

cite the reaction involved

A
  1. 2H2O to H2O2

2. 2GH-SH to GSSG (Se needed)

Biochemistry (28 decks)

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