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Biochemistry Final > Metabolism > Flashcards

Metabolism Flashcards

1
Q

Name four processes that happen in the mitochondria.

A

TCA, Ox Phos., Ac. CoA Production, Fatty Acid Oxidation

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2
Q

What processes happen in the cytoplasm?

A

Glycolysis, FA synth, Pent. Phos

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3
Q

What processes can happen in either the mitochondria or in the cytoplasm?

A

Heme synth, urea cycle, gluconeogenesis

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4
Q

Rate limiting Step of Glycolysis

A

PFK-1

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5
Q

Rate limiting Step of Gluconeogenesis

A

F-1,6-BP

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6
Q

Rate limiting Step of TCA

A

Isocitrate Dehydrogenase

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7
Q

Rate limiting Step of glycogenesis

A

Glycogen Synthase

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8
Q

Rate limiting Step of glycogenolysis

A

Glycogen Phosphorylase

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9
Q

Rate limiting Step of P.P.

A

Glucose 6 P Dehydrogenase

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10
Q

Rate limiting Step of Pyrimidine Synth

A

CPS II

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11
Q

Rate limiting Step of Purine Synth

A

PRPP

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12
Q

Rate limiting Step of Urea Cycle

A

CPS I

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13
Q

Rate limiting Step of FA Synth

A

Acetyl CoA Carboxylase (ACC)

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14
Q

Rate limiting Step of FA Oxidation

A

Carnitine Acetyltransferase I

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15
Q

Rate limiting Step of Ketogenesis

A

HMG-CoA Synthetase

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16
Q

Rate limiting Step of Cholesterol Synth

A

HMG-CoA Reductase

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17
Q

Aerobic metabolism makes ____ ATP. If you use the glycerol-3-phosphate shuttle, its net _____.

A
  1. 30.
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18
Q

Which one has a higher affinity, hexokinase or glucokinase?

A

Hexokinase

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19
Q

What reaction does Pyruvate Kinase do?

A

PEP–>Pyruvate

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20
Q

What reaction does Pyruvate DH do?

A

Pyruvate –> Acetyl CoA

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21
Q

Who makes F-2,6,-BP? What does it do?

A

Comes from Fructose 6 Phosphate with PFK-2

Promotes PFK-1 Activity (more F-1,6-BP)

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22
Q

Pyruvate Dehydrogenase requires what 5 cofactors?

A

B1, 2, 3, 5, Lipoic Acid

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23
Q

How does exercise alter your NAD+/NADH ratio?

A

Increases It

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24
Q

Pyruvate Dehydrogenase links what two pathways?

A

Glycolysis and TCA

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25
Q

How do the cofactors required for alpha-KG and pyruvate DH differ?

A

They don’t.

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26
Q

What does Arsenic do anyway?

A

Inhibits Lipoic Acid –> Loss of TPP mediated activity

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27
Q

What happens to folks with a pyruvate dehydrogenase complex deficiency? How do you treat?

A

Pyruvate Buildup –> Shunted to Lactate and Alanine.

To treat – switch to high fat diet/diet high in ketogenic nutrients (LL)

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28
Q

Four different pathways Pyruvate can take?

A

Pyruvate DH –> TCA cycle
Pyruvate Carboxylase –> Oxo
Alanine AT –> Alanine
Lactic Acid DH –> Anaerobic Glycolysis

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29
Q

Kreb’s Cycle makes _______/AcCoa

A

3 NADH, 1 FADH2, 2CO2, 1 GTP

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30
Q

How do electrons get from glycolysis into the mitochondria?

A

Malate-Aspartate or Glycerol-3-phosphate shuttle

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31
Q

Rotenone inhibits…

A

Complex 1

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32
Q

Antimycin A inhibits…

A

Complex III

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33
Q

Cyanide Inhibits…

A

Complex IV

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34
Q

CO inhibits….

A

Complex IV

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35
Q

Oligomycin inhibits….

A

Complex V (ATP Synthase)

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36
Q

1 NADH = ____ ATP. 1 FAD2 = _____ ATP.

A

2.5, 1.5

37
Q

Examples of uncoupling agents?

A

2,4 DNP, Aspirin, Thermogenin

38
Q

Who are the irreversible enzymes of Gluconeogenesis?

A

Pyruvate Carboxylase, PEP Carboxykinase, F-1,6-BP, Glu-6-Phosphotase

39
Q

Odd chain fatty acids yield _____ in metabolism.

A

Propionyl CoA

40
Q

Even chain fatty acids are a source of…

A

AcCoA Equivalent

41
Q

PP Pathway makes…

A

NADPH and Ribose

42
Q

Respiratory burst utilizes what enzyme complex?

A

NADPH Oxidase Complex

43
Q

What happens to folks with Glu-6-Phosphate dehydrogenase deficiency?

A

hemolytic anemia from diminished ability to respond to free radicals. You’ll see bite cells and heinz bodies.

44
Q

Fructose Intolerance is characterized by an insufficiency of…

A

Aldolase B

45
Q

Symptoms of fructose intolerance?

A

Hypoglycemia, jaundice, cirrhosis, vomiting

46
Q

Enzyme deficient in galactosemia?

A

Galactose-1-P-Uridyltransferase

47
Q

Symptoms of Galatosemia?

A

Failure to thrive, jaundice, hepatomegaly, cataracts

48
Q

Treatment for galactosemia?

A

Cut galactose and lactose from diet

49
Q

What is sorbitol?

A

Glucose converted to its alcohol counterpart via aldose reductase

50
Q

What converts sorbitol to fructose?

A

Sorbitol dehydrogenase

51
Q

Essential Amino Acids?

A

PVT TIM HALL

52
Q

Acidic Amino Acids?

A

Asp and Glu

53
Q

Basic AA?

A

His Lys Arg

54
Q

Point of the urea cycle?

A

Convert excess nitrogen to urea for excretion

55
Q

What AAs are important for ammonia transport?

A

Alanine and Glutamate

56
Q

Cause of hyperammonemia?

A

Too much ammonia – liver disease, enzyme deficiency

57
Q

Symptoms of hyperammonemia?

A

Excess NH4, inhibited TCA

58
Q

Why do we care about NAG deficiency?

A

required for CPS1. leads to hyperamonemia. increased ornithine

59
Q

Why do we care about ornithine transcarbamylase deficiency?

A

Most common urea disorder. X linked. Carbomoyl P –> Orotic Acid.

60
Q

What AA makes Epinephrine, NE, Dopamine, and Melanin?

A

Phenylalanine

61
Q

What AA makes Seratonin, Melatonin, and Niacin?

A

Tryptophan

62
Q

What AA makes Histamine?

A

Histidine

63
Q

What AA makes Heme?

A

Glycine

64
Q

What AA makes GABA and Glutathione?

A

Glutamate

65
Q

What AA makes Creatine, Urea, and NO?

A

Arginine

66
Q

PKU causes a failure to make what transition? What enzyme?

A

Phenylalanine –> Tyrosine

Phenylalanine Hydroxylase

67
Q

Albinism is caused by a inefficacy/absence of….

A

Tyrosinase

68
Q

How do you treat PKU?

A

Ideally – early diagnosis

less phenylalanine in diet and more tyrosine

69
Q

What causes alkaptonuria?

A

Homogenisate Oxidase

Turns urine black on prolonged air exposure. Brown sclare. May break down cartilage.

70
Q

What is homocystinuria?

A

Buildup of homocysteine.

71
Q

What is cystinuria?

A

Defect in intestinal COLA channels (cysteine, ornithine, lysine, arginine). Causes stones.

72
Q

What is maple syrup urine disease?

A

Blocked degradation of branched amino acids (leu, iso, val)

73
Q

Deficient in glucose-6-phosphatase?

A

Von Gierkes

74
Q

Deficiency in Muscle glycogen phosphorylase?

A

McArdles

75
Q

Deficient enzyme in fabry?

A

alpha-galactosidase A

76
Q

Gaucher disease enzyme deficiency?

A

Glucocerebrosidase

77
Q

Deficient enzyme in Niemann-Pick

A

Sphingomyelinase

78
Q

Deficient enzyme in Tay-Sachs

A

Hexosaminidase A

79
Q

Krabbe Disease deficient enzyme,

A

Galactocerebrosidase.

80
Q

Fastest way to identify something as hunter’s or hurler’s?

A

Heparan sulfate build up.

81
Q

Who helps transport long-chain fatty acids into the mitochondrial matrix?

A

Carnitine.

82
Q

So carnitine deficiency…what happens there.

A

Can’t transport LCFAs into the mitchondria. Toxic accumulation.

83
Q

In alcoholics, increased NADH converts oxo into…

A

Malate

84
Q

In starvation – what points everyone toward ketosis?

A

Gluconeogenesis uses up all of the Oxo.

85
Q

In people in ketosis…what does their breath smell like?

A

Fruity

86
Q

What does HMG-CoA reductase do….really?

A

Converts HMG-CoA to melvalonate.

87
Q

Who degrades TG in adipocytes?

A

Hor-sensitive lipase

88
Q

What does lipoprotein lipase do?

A

Degrades TG in chylomicrons and VLDL

Biochemistry Final (1 decks)

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