✅ BIOCHEM - Metabolic Pathways Flashcards

1
Q

Example of an amphibolic pathway

A

Krebs

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2
Q

Where does glycolysis occur?

A

Cytoplasm

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3
Q

Substrate of glycolysis

A

Glucose

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4
Q

End product of glycolysis

A

Pyruvate or lactate

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5
Q

Rate limiting step of glycolysis

A

Fructose 6 phosphate -> fructose 1,6 bisphosphate

Enzyme: PFK-1

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6
Q

What type of glycolysis happens in cells with mitochondria and adequate O2?

A

Aerobic glycolysis

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7
Q

2 stages of glycolysis

A

Energy investment and energy generation

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8
Q

3 important steps in glycolysis

A

Step 1
Step 3
Step 10

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9
Q

NADH generating step in glycolysis

A

Glyceraldehyde 3 phosphate –> 1,3 bisphosphoglycerate

Enzyme: glyceraldehyde phosphate dehydrogenase

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10
Q

Which has a lower km and comsequently higher affinity?

Hexokinase or glucokinase?

A

Hexokinase

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11
Q

Glucokinase is present only in what type of tissues

A

Liver

Islet cells of pancreas

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12
Q

Hexokinase has a higher Vmax than glucokinase. True or false.

A

False

Glucokinase has a higher Vmax

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13
Q

Hexokinase is inhibited by

A

Glucose 6 phosphate

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14
Q

Glucokinase is inhibited by

A

Fructose 6 phosphate

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15
Q

Glucokinase liver activity is induced by

A

Insulin

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16
Q

Action of PFK-2

A

Converts fructose 6 phosphate to fructose 2,6 BP

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17
Q

Inhibitor of PFK-1

A

ATP and Citrate

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18
Q

Inhibitor of PFK 2

A

Starved state

Decrease insulin and inc glucagon

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19
Q

2 steps in glycolysis that produce ATP via substrate level phosphorylation

A

1,3 BPG to 3 PG
Enzyme: phosphoglycerate kinase

PEP to pyruvate
Enzyme: pyruvate kinase

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20
Q

2 outcomes of pyruvate in glycolysis

A

Can enter the citric acid cycle in aerobic glycolysis

Can be reduced to lactate in anerobic glycolysis

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21
Q

NADH produced in glycolysis cannot pass through thr mitochondrial membrane so it needs what?

A

Malate aspartate shuttle

Glycerol phosphate shuttle

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22
Q

Malate aspartate shuttle is used in what organs?

A

Liver kidney heart

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23
Q

Glycerol phosphate shuttle is used in which organs?

A

Skeletal muscle

Brain

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24
Q

Kinds of metabolic pathways

A

Anabolic
Catabolic
Amphibolic

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25
Q

NADH in the malate aspartate shuttle will yield how many ATP?

A

3

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26
Q

NADH in the glycerol phosphate shuttle will yield how many atp?

A

2

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27
Q

In what part of the cell can u find the ETC?

A

Inner mitochondrial membrane

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28
Q

What happens to pyruvate in anaerobic glycolysis?

A

Lactate dehydrogenase uses up NADH and converts pyruvate to lactate.

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29
Q

Anaerobic glycolysis strictly happens in which organs?

A
Cornea
Lens
Kidney medulla
Wbc 
Rbc
Testes
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30
Q

Enzyme responsible for conversion of 1,3 BPG to 2,3 BPG

A

Bisphosphoglycerate mutase

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31
Q

The Luebering-Rapoport pathway bypasses what enzyme

A

Phosphoglycerate kinase

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32
Q

Most common enzyme defect in glycolysis

A

Pyruvate kinase deficiency

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33
Q

Characteristic peripheral smear of G6PD deficiency

A

Heinz bodies

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34
Q

Most common precipitating history of G6PD

A

Infection

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35
Q

Pyruvate is converted to ethanol by

A

Pyruvate carboxylase (yeasts)

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36
Q

Pyruvate is converted to oxaloacetate by

A

Pyruvate carboxylase (gluconeogenesis)

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37
Q

What is the mechanism in arsenic poisoning?

A

Arsenic inhibits pyruvate dehydrogenase by binding to LIPOIC ACID

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38
Q

Pyruvate is converted to acetyl Coa by

A

Pyruvate dehydrogenase complex

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39
Q

Coenzymes of pyruvate dehydrogenase

A
Thiamine pyrophosphate
FAD
NAD+
Coenzyme A (Pantothenic Acid)
Lipoic Acid
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40
Q

Pyruvate kinase deficiency usually manifests clinically as

A

Chronic hemolytic anemia

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41
Q

Products of conversion of pyruvate to acetyl coa

A

Acetyl coa
NADH
CO2

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42
Q

Most common cause of congenital lactic acidosis

A

Pyruvate dehydrogenase deficiency

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43
Q

Pattern of inheritance of pyruvate kinase deficiency

A

X-linked DOMINANT

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44
Q

Treatment for pyruvate dehydrogenase deficiency

A

Ketogenic diet

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45
Q

Rationale for ketogenic diet in pyruvate dehydrogenase deficiency

A

Increase lysine and leucine to bypass glycolysis

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46
Q

Mechanism of pyruvate dehydrogenase deficiency in chronic alcoholism.

A

Thiamine deficiency (acquired)

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47
Q

Where does the Kreb’s cycle occur?

A

Mitochondrial matrix

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48
Q

TCA occurs in the mitochondrial matrix except for which process?

A

Succinate to fumarate

Enzyme: succinate dehydrogenase

49
Q

Rate limiting step in krebs

A

Isocitrate to alpha ketoglutarate

Enzyme: isocitrate dehydrogenase

50
Q

Inhibitor of aconitase

A

Fluoroacetate (rat poison)

51
Q

Inhibitor of alpha ketoglutarate dehydrogenase

A

Arsenite

52
Q

Process in the TCA that produces GTP

A

Succinyl Coa to succinate

Enzyme: succinate thiokinase

53
Q

Citrate delivers acetyl Coa to the cytoplasm for ______ via the _______

A

Fatty acid synthesis via the citrate shuttle

54
Q

Succinyl coa in TCA is also involved in _______

A

Heme synthesis

Activation of ketone bodies in extrahepatic tissues

55
Q

Malate in TCA is also use for

A

Gluconeogenesis

56
Q

What is the main function of gluconeogenesis

A

Prevent hypoglycemia

57
Q

What is the main function of the Cori cycle?

A

Conversion of lactate to glucose

58
Q

Gluconeogenesis occurs in which organs

A

Liver 60% and kidney 10%

Prolonged fasting: kidney up to 40%

59
Q

Gluconeogenesis occur in the

A

Cytoplasm and mitochondria

60
Q

Rate limiting step in gluconeogenesis

A

Fructose 1,6 bisphosphate to fructose 6 phosphate

Enzyme: fructose 1,6 bisphosphatase

61
Q

Energy expense in the cori cycle

A

4 ATPs

62
Q

In glucogeonesis pyruvate is converted back to PEP through which of the following steps?

A

Pyruvate to OAA
Enzyme: Pyruvate carboxylase

OAA to PEP
Enzyme: PEP Carboxykinase

63
Q

All carboxylases require _____ as a cofactor

A

Biotin

64
Q

PEP carboxykinase require ____

A

GTP

65
Q

Inhibitor of fructose 1,6 BP to fructose 6-P in gluconeogenesis

A

Fructose 2,6 BP and AMP

66
Q

Energy expenditure in gluconeogenesis

A

4Atps
2 gtps
2 NADH to NAD+

67
Q

Glucosuria occurs when venous blood glucose concentration exceeds

A

9.5-10 mmol/L (renal threshold)

180 g/dl

68
Q

Redness when drinking is due to which enzyme

A

Acetaldehyde dehydrogenase

69
Q

High amounts of NADH favors the ff reactions

A

Pyruvate to lactate
OAA to malate
DHAP to glycerol 3 phosphate

70
Q

Insulin resistance in pregnancy is due to

A

Human placental lactogen

71
Q

Hyperinsulinemia in pregnancy is due to

A

Increased estrogen levels

72
Q

Major carbohydrate storage in animals

A

Glycogen

73
Q

Processes that happen in the cytoplasm and mitochondria

A

Gluconeogenesis
Urea synthesis
Heme synthesis

74
Q

Glycogen stores are used up after

A

12-18 hours

75
Q

Glycogen is stored in which 2 organs?

A

Liver and muscle

76
Q

Activated form of glucose

A

UDP-glucose

77
Q

Synthesis of new glycogen molecules from alpha-d-glucose

A

Glycogenesis

78
Q

Rate limiting step in glycogenesis

A

Elongation of glycogen chains

Enzyme: glycogen synthase

79
Q

Enzyme involved in conversion of glucose 6 phosphate to glucose 1 phosphate

A

Phosphoglucomutase

80
Q

Enzyme involved in the synthesis of UDP glucose

A

UDP glucose phosphorylase

81
Q

Formation of branches in glycogen is mediated by what enzyme

A

Branching enzyme (amylo and transglucosidase)

82
Q

Glycogen synthase is to glycogenesis

_____ is to glycogenolysis

A

Glycogen phosphorylase

83
Q

Glycogen phosphorylase requires what coenzyme

A

Pyridoxal phosphate

84
Q

Glycogen phosphorylase cannot cleave a 4 glucose residue before a branch called the

A

Limit dextrin

85
Q

Rate limiting step in glycogenolysis

A

Removal of glucose using glycogen phosphorylase

86
Q

Lysosomal degradation of glycogen happens with what enzyme

A

Alpha 1,4 glucosidase

87
Q

Other name of alpha 1,4 glucosidase

A

Acid maltase

88
Q

In glycogen phosphorylase, the active form is the phosphorylated or dephosphorylated?

A

Phosphorylated

89
Q

Deficiency in acid maltase

A

Pompe disease

90
Q

Deficiency in glucose 6 phosphatase

A

Von gierke disease

91
Q

Deficiency in skeletal muscle glycogen phosphorylase

A

McArdle’s disease

92
Q

Deficient in Andersen’s disease

A

Branching enzyme

93
Q

Cori’s disease deficiency

A

Debranching enzyme

94
Q

Deficient PFK

A

Tarui’s disease

95
Q

Major dietary source of galactose

A

Lactose

96
Q

Activated form of galactose

A

UDP galactose

97
Q

Glucose 1 phosphate + UDP glucose forms _____ through which enzyme

A

UDP galactose and glucose 1 phosphate

Enzyme: galactose 1 P uridyl transferase

98
Q

UDP galactose –> UDP galactose

Enzyme?

A

UPD hexose 4 epimerase

99
Q

Main products of the pentose phosphate pathway

A

NADPH

Ribose 5 phosphate

100
Q

Substrate of the pentose phosphate pathway

A

Gucose 6 phosphate

101
Q

The pentose phosphate pathway is divided into the

A

Oxidative and non oxidative phase

102
Q

Main product of the oxidative phase of the PPP

A

NADPH

Ribulose 5 P

103
Q

Phase 1 of PPP is

A

Oxidative and irreversible

104
Q

Key enzyme in oxidative phase of pentose phosphate pathway

A

G6PD

105
Q

Key enzymes in non-oxidative phase of the pentose phosphate pathway

A

Transketolases

106
Q

Transketolases require

A

Thiamine

107
Q

Rate limiting step in the pentose phosphate pathway

A

Glucose 6 P to 6 phosphogluconate

Enzyme: G6PD

108
Q

Reduced glutathione is recreated using

A

NADPH

Enzyme: glutathione REDUCTASE

109
Q

Reduced glutathione sequestere harmful H2O2 using

A

Glutathione peroxidase

110
Q

Drugs that precipitate G6PD deficiency

A

AAA

Antibiotics (sulfonamides, chloramphenicol)
Antimalarials (primaquine)
Antipyretics (except ASA and Paracetamol)

111
Q

Chronic granulomatous disease is secondary to to deficiency in

A

NADPH OXIDASE

112
Q

Chronic granulomatous disease is caused by what type of bacteria

A

Catalase positive

113
Q

Sorbitol dehydrogenase is found in

A

The seminal vesicles only

Catalyzes sorbitol to fructose (main sperm energy source)

114
Q

Glucose to sorbitol

What enzyme?

A

Aldose reductase

115
Q

Mannise is an important component in

A

Glycoproteins

116
Q

How is mannose 6 P used?

A

Mannose 6 P is converted to fructose 6 P by

Phosphomannose isomerase

117
Q

Fructose 1 P to dihydroxyacetone phosphate

What enzyme?

A

Aldolase B

118
Q

Fructose to fructose 1 P

A

Fructokinase or hexokinase