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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
[Phosphorylation/ Dephosphorylation] glycogen synthase when active
dephosphorylated
26
[Phosphorylation/ Dephosphorylation] glycogen phosphorylase when active
phosphorylated
27
[Lysosomal Storage disease] glucose 6 phosphatase deficiency
Von Gierke Ia
28
[Lysosomal Storage disease] lysosomal alpha glucosidase
Pompe II
29
[Lysosomal Storage disease] debranching enzyme
Cori III
30
[Lysosomal Storage disease] branching enzyme
Andersen IV
31
[Lysosomal Storage disease] muscle phosphorylase
McArdle V
32
[Lysosomal Storage disease] liver phosphorylase
Hers disease VI
33
[Lysosomal Storage disease] Increased glycogen in liver and renal tubule, hypoglycemia lactic acidosis, ketosis hyperlipemia
Von Gierke
34
[Lysosomal Storage disease] increase in glycogen lysosome hypotonia, death from heart failure by age 2
Pompe
35
[Lysosomal Storage disease] fasting hypoglycemia hepatomegaly in infancy increase in limit dextrin muscle weakness
Cori disease debranching enzyme
36
[Lysosomal Storage disease] hepatosplenomegaly increasepolysaccharide with few branch points, death from heart failure before age 5
Andersen branching enzyme
37
[Lysosomal Storage disease] poor exercise tolerance, muscle cramps, and myoglobinuria but no lactic acidosis, increase muscle glycogen
Mcardle disease
38
[Lysosomal Storage disease] hepatomegaly, mild hypoglycemia
Hers disease
39
[Galactose Metabolism] enzyme needed to convert Galactose to Galactose 1 phosphate
Galactokinase
40
[Galactose Metabolism] enzyme needed to convert Galactose1P to UDP galactose
UDP-hexose 4 epimerase
41
[Diagnose] galactosemia + galactusuria cataracts in early childhood
galaktokinase
42
[Diagnose] galactosemia, galactosuria, cataracts, jaundice, vomiting, diarrhea poor growth, severe mental retardation, liver damage premature ovarial failure
Classic Galactosemia Galactose1-phosphate uridyl transferase deficiency
43
[Fructose Metabolism] What is the enzyme needed to convert Fructose to Fructose-1-P?
Fructokinase or Hexokinase
44
[Fructose Metabolism] What is the enzyme needed to convert Fructose1P to DHAP + glyceraldehyde?
Aldolase B
45
[Fructose Metabolism] Aldolase B is found in what body organ?
Liver
46
[Fructose Metabolism] Aldolase C is found in what organ?
brain
47
[Fructose Metabolism] What type of aldolase that is present in most tissues?
Aldosalse A
48
[Fructose Metabolism] Enzyme involved in asymptomatic patients but with fructosuria
Fructokinase deficiency
49
[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
aldolase B deficiency
50
What pathway is used when glucose is converted to sorbitol
polyol pathway Enzyme: Aldose reductase
51
[Polyol pathway] What is the enzyme essential to convert glucose to sorbitol?
Aldose reductase needs NADPH + H+
52
[Polyol pathway] What enzyme is needed to convert sorbitol to fructose
Sorbitol dehydrogenase
53
what part of the body has no sorbitol dehydrogenase?
1. Retina 2. Lens 3. Kidney 4. Schwann cells
54
Sorbitol dehydrogenase are found in what organs?
1. Liver 2. Ovaries 3. Seminal vesicles
55
[Diagnosis] adult african or asian descent flatulence and diarrhea after ingestion of dairy products
Lactose intolerance | Lactase deficiency
56
[Diagnosis] intolerance after ingestion of sugar Inuit people of greenland and canada
sucrase isomaltase complex deficiency
57
What pathway is an alternative pathway for oxidation of glucose in the liver?
uronic acid pathway
58
What is the main pathway for production of glucoronic and iduronic acid
uronic acid pathway
59
What glucose derivative is an essential component of glycosaminoglycans?
glucoronic acid
60
Glucoronic acid is required in detoxification reactions of this compounds
1. Bilirubin 2. Steroids 3. Morphine and other drugs
61
What enzyme is implicated in primates' inability to synthesize vitamin C
L-gulunolactose oxidase
62
[Uronic pathway] What converts UDP glucose to UDP glucoronic acid?
UDP Glucose dehydrogenase
63
[Diagnose] increased xylulose in the urine; benign with no clinical consequence
Xylulose reductase deficiency
64
What is the main pathway produces NADPH?
Pentose Phosphate Pathway
65
Ribose 5-phosphate is produced in what pathway
Hexose Monophosphate Shunt (Pentose Phosphate Pathway) to use 5-carbon sugar
66
[Pentose Phosphat Pathway] What is its substrate?
Glucose-6-phosphate
67
[Pentose Phosphat Pathway] What is the rate limiting step
Glucose6Phosphate to 6phosphogluconate via Glucose 6 phosphate dehydrogenase
68
[Pentose Phosphat Pathway] What are the products of the oxidative, irreversible phase
Enzyme: G6P dehydrogenase pdt: 2NADPH +R5P
69
[Pentose Phosphat Pathway] what are the products of the second, reversible phase?
Enzyme: transketolase, Transaldolase Pdts: 1. R5P 2. F6P 3. G3P 4. Other CHO
70
[Pentose Phosphat Pathway] what is the cofactor of transketolase?
Thiamine
71
[Pentose Phosphate Pathway: Clinical correlate] Glutathione peroxidase removes what cellular byproduct?
H2O2
72
What enzyme is the most common disease producing enzyme abnormality in humans?
G6PD deficiency
73
What is the role of gluthathione reductase? | Cite the reaction involved
1. NADPH + NADP | 2. GSSG to 2G-SH (FAD needed)
74
What is the role of glutathione peroxidase? | cite the reaction involved
1. 2H2O to H2O2 | 2. 2GH-SH to GSSG (Se needed)

Biochemistry (28 decks)

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