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Biochemistry Final > Metabolism > Flashcards

Metabolism Flashcards

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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
How do the cofactors required for alpha-KG and pyruvate DH differ?
They don't.
26
What does Arsenic do anyway?
Inhibits Lipoic Acid --> Loss of TPP mediated activity
27
What happens to folks with a pyruvate dehydrogenase complex deficiency? How do you treat?
Pyruvate Buildup --> Shunted to Lactate and Alanine. To treat -- switch to high fat diet/diet high in ketogenic nutrients (LL)
28
Four different pathways Pyruvate can take?
Pyruvate DH --> TCA cycle Pyruvate Carboxylase --> Oxo Alanine AT --> Alanine Lactic Acid DH --> Anaerobic Glycolysis
29
Kreb's Cycle makes _______/AcCoa
3 NADH, 1 FADH2, 2CO2, 1 GTP
30
How do electrons get from glycolysis into the mitochondria?
Malate-Aspartate or Glycerol-3-phosphate shuttle
31
Rotenone inhibits...
Complex 1
32
Antimycin A inhibits...
Complex III
33
Cyanide Inhibits...
Complex IV
34
CO inhibits....
Complex IV
35
Oligomycin inhibits....
Complex V (ATP Synthase)
36
1 NADH = ____ ATP. 1 FAD2 = _____ ATP.
2.5, 1.5
37
Examples of uncoupling agents?
2,4 DNP, Aspirin, Thermogenin
38
Who are the irreversible enzymes of Gluconeogenesis?
Pyruvate Carboxylase, PEP Carboxykinase, F-1,6-BP, Glu-6-Phosphotase
39
Odd chain fatty acids yield _____ in metabolism.
Propionyl CoA
40
Even chain fatty acids are a source of...
AcCoA Equivalent
41
PP Pathway makes...
NADPH and Ribose
42
Respiratory burst utilizes what enzyme complex?
NADPH Oxidase Complex
43
What happens to folks with Glu-6-Phosphate dehydrogenase deficiency?
hemolytic anemia from diminished ability to respond to free radicals. You'll see bite cells and heinz bodies.
44
Fructose Intolerance is characterized by an insufficiency of...
Aldolase B
45
Symptoms of fructose intolerance?
Hypoglycemia, jaundice, cirrhosis, vomiting
46
Enzyme deficient in galactosemia?
Galactose-1-P-Uridyltransferase
47
Symptoms of Galatosemia?
Failure to thrive, jaundice, hepatomegaly, cataracts
48
Treatment for galactosemia?
Cut galactose and lactose from diet
49
What is sorbitol?
Glucose converted to its alcohol counterpart via aldose reductase
50
What converts sorbitol to fructose?
Sorbitol dehydrogenase
51
Essential Amino Acids?
PVT TIM HALL
52
Acidic Amino Acids?
Asp and Glu
53
Basic AA?
His Lys Arg
54
Point of the urea cycle?
Convert excess nitrogen to urea for excretion
55
What AAs are important for ammonia transport?
Alanine and Glutamate
56
Cause of hyperammonemia?
Too much ammonia -- liver disease, enzyme deficiency
57
Symptoms of hyperammonemia?
Excess NH4, inhibited TCA
58
Why do we care about NAG deficiency?
required for CPS1. leads to hyperamonemia. increased ornithine
59
Why do we care about ornithine transcarbamylase deficiency?
Most common urea disorder. X linked. Carbomoyl P --> Orotic Acid.
60
What AA makes Epinephrine, NE, Dopamine, and Melanin?
Phenylalanine
61
What AA makes Seratonin, Melatonin, and Niacin?
Tryptophan
62
What AA makes Histamine?
Histidine
63
What AA makes Heme?
Glycine
64
What AA makes GABA and Glutathione?
Glutamate
65
What AA makes Creatine, Urea, and NO?
Arginine
66
PKU causes a failure to make what transition? What enzyme?
Phenylalanine --> Tyrosine | Phenylalanine Hydroxylase
67
Albinism is caused by a inefficacy/absence of....
Tyrosinase
68
How do you treat PKU?
Ideally -- early diagnosis | less phenylalanine in diet and more tyrosine
69
What causes alkaptonuria?
Homogenisate Oxidase Turns urine black on prolonged air exposure. Brown sclare. May break down cartilage.
70
What is homocystinuria?
Buildup of homocysteine.
71
What is cystinuria?
Defect in intestinal COLA channels (cysteine, ornithine, lysine, arginine). Causes stones.
72
What is maple syrup urine disease?
Blocked degradation of branched amino acids (leu, iso, val)
73
Deficient in glucose-6-phosphatase?
Von Gierkes
74
Deficiency in Muscle glycogen phosphorylase?
McArdles
75
Deficient enzyme in fabry?
alpha-galactosidase A
76
Gaucher disease enzyme deficiency?
Glucocerebrosidase
77
Deficient enzyme in Niemann-Pick
Sphingomyelinase
78
Deficient enzyme in Tay-Sachs
Hexosaminidase A
79
Krabbe Disease deficient enzyme,
Galactocerebrosidase.
80
Fastest way to identify something as hunter's or hurler's?
Heparan sulfate build up.
81
Who helps transport long-chain fatty acids into the mitochondrial matrix?
Carnitine.
82
So carnitine deficiency...what happens there.
Can't transport LCFAs into the mitchondria. Toxic accumulation.
83
In alcoholics, increased NADH converts oxo into...
Malate
84
In starvation -- what points everyone toward ketosis?
Gluconeogenesis uses up all of the Oxo.
85
In people in ketosis...what does their breath smell like?
Fruity
86
What does HMG-CoA reductase do....really?
Converts HMG-CoA to melvalonate.
87
Who degrades TG in adipocytes?
Hor-sensitive lipase
88
What does lipoprotein lipase do?
Degrades TG in chylomicrons and VLDL

Biochemistry Final (1 decks)

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