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Biochemistry > Carbohydrates > Flashcards

Carbohydrates Flashcards

1
Q

Rate limiting step of glycolysis

A

F6P -> F1,6P

Phosphofructokinase 1

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2
Q

NADPH producing reaction in glycolysis

A

G3P -> 1,3-BPG

G3P dehydrogenase

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3
Q

Shuttle in liver, kidney, heart for NADH

A

Malate-Aspartate Shuttle

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4
Q

NADH shuttle in skeletal muscle and brain

A

Glycerol phosphate shuttle

1 NADH: 2 ATP

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5
Q

Strictly glycolytic organs (produces lactate)

A 
RBCs
Lens
Cornea
Kidney medulla
Testis
WBCs
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6
Q

Arsenic poisoning affects which enzymes?

A

Pryruvate dehydrogenase

Glyceraldehyde 3 phosphate dehydrogenase (NADH producing step)

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7
Q

Most common enzymatic defect in glycolysis?

A

Pyruvate kinase deficiency
For last step: PEP-> pyruvate
Sx: chronic hemolytic anemia

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8
Q

Pyruvate dehydrogenase cofactors?

A

Pyruvate-> acetyl CoA

Thiamine pyrophosphate
FAD
NADH
coenzyme A (contains pantothenic acid)
Lipoid acid
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9
Q

Where does TCA cycle occur?

A

Mitochondrial matrix

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10
Q

Pasaway enzyme that stays in the IMM while everyone is in the matrix?

A

Succinate dehydrogenase

Succinate -> fumarate

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11
Q

Rate limiting step in TCA cycle?

A

Isocitrate -> a-ketoglutarate

Isocitrate dehydrogenase

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12
Q

What enzyme does rat kill (fluoroacetate) affect?

A

Aconitase

Citrate-> isocitrate

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13
Q

GTP is produced in what reaction?

A

Succinyl CoA -> succinate
Succinate thiokinase

Ang Gatas Galing sa SU-SU.

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14
Q

NADH is produced in which steps of the TCA cycle?

A

Isocitrate -> a-ketoglutarate
a-ketoglutarate -> succinyl CoA
Malate-> oxaloacetate

  • pyruvate -> acetyl CoA
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15
Q

FADH2 is produced in which TCA step?

A

Succinate -> fumarate

Succinate dehydrogenase

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16
Q

Which TCA intermediates can be redirected somewhere else?

A

Citrate
Succinyl CoA
Malate

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17
Q

ATP yield from TCA cycle (from pyruvate)

A

15!! (X2 if counting per glucose molecule)

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18
Q

Where does gluconeogenesis occur?

A

Liver (90%) and Kidneys (10%)

Mitochondria and cytoplasm

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19
Q

Cori cycle is for?

A

Lactate from muscles -> glucose
Occurs at the liver
Uses 4 ATP

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20
Q

What cofactor is needed in carboxylase enzymes? (Pyruvate carboxylase)

A

Biotin!

Pyruvate carboxylase
Acetyl CoA carboxylase
Propionyl CoA carboxylase

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21
Q

Rate limiting step for gluconeogenesis?

A

F1,6BP -> F6P

Fructose 1,6 bisphosphatase

22
Q

What enzyme is present only in liver and kidneys, hence limiting gluconeogenesis in these organs?

A

Glucose 6-phosphatase

23
Q

High NADH levels due to chronic alcohol use favor which reactions?

A

Pyruvate to lactate
OAA to Malate
DHAP to glycerol 3 phosphate

24
Q

Glycosidic bond for branching?

A

a (1->6)

25
Q

Glycosidic bonds for elongation?

A

a (1->4)

26
Q

Where does glycogenesis occur?

A

Liver, muscle

Cytosol

27
Q

Core protein for glycogenesis?

A

Glycogenin

28
Q

Rate limiting step for glycogenesis?

A

Elongation of glycogen

Glycogen synthase

29
Q

Glycogen stores last for how long?

A

12-18 hours

30
Q

Rate limiting step for glycogenolysis?

A 
Glycogen phosphorylase
Requires pyridoxal phosphate
Breaking a(1->4) bonds
31
Q

Type I glycogen storage disease

A

Von Gierke’s
Glucose 6-phosphatase
Glycogen in liver and kidneys, hypogly, lactic acidosis, kerosine

32
Q

Glycogen storage disease that causes accumulation of glycogen in lysosomes, cardiomegaly, and heart failure?

A

Type II
Pompe’s disease
Acid maltase deficiency

33
Q

Deficiency in debranching enzyme that causes mild hypoglycemia?

A

Type III

Cori’s disease

34
Q

Glycogen storage disease that causes severe form of type I (early death from heart and liver failure). Deficiency in branching enzyme.

A

Type IV

Andersen’s disease

35
Q

Problem with skeletal muscle glycogen phosphorylase causing muscle cramps and myoglobinuria but no lactic acidosis

A

Type V

McArdle’s disease

36
Q

Type VI or Her’s disease is a deficiency in what is enzyme?

A

Hepatic glycogen phosphorylase

Causes glycogen in liver cells and hypoglycemia

37
Q

Deficiency in phosphofructokinase causing manifestations similar to type V glycogen storage disease but with hemolytic anemia

A

Type VII

Tarui’s disease

38
Q

Type VIII glycogen storage disease is a deficiency in what enzyme?

A

Hepatic phosphorylase kinase

Presents like her’s disease (glycogen in liver cells, hypogly)

39
Q

Autosomal recessive disease that causes cataracts + hepatosplenomegaly + mental retardation

A

Classic galactosemia
Deficiency in galactose 1-P uridyltransferase
Accumulation of galactitol

40
Q

Differentiate aldolase A and B

A

Aldolase A: glycolysis
F1,6BP to DHAP + glycerol 3-P

Aldolase B: fructose metabolism
F1P -> DHAP + glyceraldehyde

41
Q

Deficienct enzyme in essential fructose is

A

Fructokinase

Benign and asymptomatic

42
Q

Deficiency in aldolase B causes?

A

Fructose intolerance
Autosomal recessive

Sx: hypogly, jaundice, cirrhosis, vomiting

43
Q

Enzyme that converts glucose to sorbitol?

A

Aldose reductase

44
Q

Sorbitol dehydrogenase is found only in?

A

Seminal vesicles

Converts sorbitol to fructose

45
Q

Glycolysis: where?

A

Cytoplasm of all cells

46
Q

Main products of pentose phosphate pathway?

A

NADPH
Ribose 5 phosphate
Glyceraldehyde 3 phosphate
Fructose 6 phosphate

47
Q

Rate limiting step in PPP?

A

Glucose 6 phosphate dehydrogenase

G6P -> 6-phosphogluconate

48
Q

Enzymes for phase 2 of PPP? What vitamin is required?

A

Transketolases

B1- thiamine

49
Q

Explain the problem in G6PD deficiency

A

Lack of NADPH inhibits action of glutathione reductase.
Glutathione peroxidase cannot break down H2O2
Accumulation of free radicals and peroxides

Hemolytic anemia triggered by oxidative stress

50
Q

Chronic granulomatous disease is a deficiency in what enzyme?

A

NADPH Oxidase
No superoxides formed for respiratory burst
Severe, persistent and chronic pyogenic infections with catalase + bacteria

Biochemistry (5 decks)

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