Metabolism I Flashcards

1
Q

beta ox location

A

mitochondria

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2
Q

TCA cycle location

A

mitochondria

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3
Q

ox phos location

A

mito

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4
Q

HMP shunt location

A

cytoplasm

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5
Q

glycolysis location

A

cytoplasm

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6
Q

kinase

A

add P using ATP

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7
Q

phosphorylase

A

add P without using ATP

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8
Q

phosphatase

A

remove P

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9
Q

dehydrogenase

A

catalyze redox rxn

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10
Q

hydroxylase

A

add OH group

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11
Q

carboxylase

A

transfer CO2 group

require biotin

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12
Q

mutase

A

relocate functional group

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13
Q

glycolysis rate limiting step

A

PFK-1

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14
Q

gluconeogenesis rate limiting step

A

fructose 1,6 bisphosphatase

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15
Q

TCA cycle rate limiting step

A

isocitrate DH

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16
Q

glycogenolysis rate limiting step

A

glycogen phosphorylase

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17
Q

HMP shunt rate limiting step

A

G6PD

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18
Q

de novo pyrimidine synthesis rate limiting step

A

CPS II

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19
Q

de novo purine synthesis rate limiting step

A

PRPP amidotransferase

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20
Q

urea cycle rate limiting step

21
Q

fatty acid synthesis rate limiting step

A

acetyl CoA carboxylase

ACC

22
Q

fatty oxidation rate limiting step

A

carnitine acyltransferase I

23
Q

ketogenesis rate limiting step

A

HMG CoA synthase

24
Q

choletserol synthesis rate limiting step

A

HMG-CoA reductase

25
Q

fructose 2,6 bisphoshate regulation

A

stimulate PFK-1 - glycolysis

inhibit fructose 1,6 - bisphosphatase - gluconeogenesis

26
Q

regulation isocitrate dehydrogenase

A

rate limiting step - TCA cycle

inhibitor - ATP and NADH
stimulation - ADP

27
Q

malate aspartate shuttle

A

heart and liver

produce 32 ATP from glucose

28
Q

glycerol-3 phosphate shuttle

A

muscle

produce 30 ATP from glucose

29
Q

arsenic

A

cause glycolysis produce 0 ATP

30
Q

vit B3

A

source of NAD+

31
Q

vit B2

A

source of FAD+

32
Q

NADPH production

33
Q

hexokinase

A

most tissue - not liver and beta cells

low Km
low Vmax
feedback inhibition - glucose 6 P

34
Q

mutation with maturity onset diabetes of young

A

glucokinase

35
Q

glucokinase

A

liver and pancreatic beta cells

high Km
high Vmax

induced by insulin

36
Q

hexokinase vs. glucokinase

A

low glucose - hexokinase sequester in tissue

high glucose - glucokinase store in liver

37
Q

fructose 2,6 biphosphate regulation

A

FBP-ase 2 and PFK-2 - part of same enzyme

  • PFK-2 produces fructose 2,6 biphosphate (stimulate PFK-1)
  • FBPase-2 produces fructose 6-P (gluconeogenesis)

fasting state - glucagon - enzyme P - FBP-ase 2 activity - more gluconeogenesis

fed state - insulin - enzyme de-P - PFK-2 activity - more glycolysis

38
Q

pyruvate dehydrogenase

A

links glycolysis and TCA cycle
-active in fed state

pyruvate > acetyl CoA

requires cofactors - TLCFN

39
Q

TLCFN

A

cofactors

thiamine -B1
lipoic acid
CoA - B5
FAD - riboflavin - B2
NAD - niacin - B3

necessary for

  • pyruvate DH
  • a-KG DH - TCA cycle
40
Q

increased serum alanin, neuro deficit, lactic acidosis

A

deficient pyruvate DH complex

build up of pyruvate - shunted to lactate and alanine production

41
Q

tx pyruvate DH deficiency

A

high fat diet
high lysine and leucine - ketogenic AAs

low glucose

42
Q

pyruvate has what possible pathways

A

alanine
OAA
acetyl CoA
lactate

43
Q

irreversible enzymes of TCA cycle

A

citrate synthase
isocitrate DH - rate limiting
a-KG DH

44
Q

products of TCA cycle

A

3 NADH
1 FADH2
2 CO2
1GTP

occurs 2x - 2 acetyl CoA formed per glucose

45
Q

TCA cycle location

A

mitochondria

46
Q

pyruvate carboxylase

A

pyruvate > OAA

47
Q

pyruvate dehydrogenase

A

pyruvate > acetyl CoA

48
Q

alanine aminotransferase

A

pyruvate > alanine

49
Q

lactic acid dehydrogenase

A

pyruvate > lactate