quiz 3 Flashcards

1
Q

Anaplerotic reactions

A

reactions that form intermediates in a metabolic pathway like TCA but aren’t from precursors

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2
Q

Nutrients for Fats to Acetyl CoA

A

Carnitine

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3
Q

Nutrients for Carbs to Acetyl CoA

A

B1, B2, B3, B5 and lipoic acid

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4
Q

Nutrients for proteins to Acetyl CoA

A

B1, B2, B3, B5 and lipoic acid

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5
Q

Nutrients required for TCA cycle (1 AA, 3 minerals, 5 vits)

A

cystein, fe, mg, mn, b1,b2,b3,b5 lipoic acid

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6
Q

Anaplerotic — makes fumarate

A

Tyr, phe

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7
Q

Anaplerotic makes succinyl CoA

A

Leu, lle, val, met

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8
Q

Anaplerotic makes A-KG

A

Glu, his, arg, pro, gln

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9
Q

Anaplerotic makes OAA

A

Asp

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10
Q

TCA cycle leads to accumulation of

A

NADH and FADH2

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11
Q

ETC cytochromes require

A

CoQ10

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12
Q

What nutrient is required to synthesize CoA

A

B5

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13
Q

Fatty acyl-co A esters are transferred across the mitochondrial membrane via

A

carnitine shuttle

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14
Q

Carnitine shuttle is upregulated by CGD

A

carnitine, genistein, daidzein

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15
Q

Adipate is a __ carbon FA

A

6

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16
Q

Suberate is a __ carbon FA

A

8

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17
Q

Increase is adipate and suberate are due to (2)

A

leakage from peroxisomes, low b2

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18
Q

Treatment of increased adipate and suberate (2)

A

glycine and carnitine

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19
Q

Nutrients to lower ethylmalonate

A

carnitine and FAD,

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20
Q

Low fatty acid oxidation leads to accumulation of urinary

A

adipate, suberate, ethylmalonate

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21
Q

Carbohydrate metabolism markers (4)

A

lactate, pyruvate, B-hydroxybutyrate, oxalate

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22
Q

Anaerobic products of glycolysis

A

pyruvate —> lactate

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23
Q

Aerobic products of glycolysis

A

Pyruvate–> acetyl coA

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24
Q

Co Factors for pyruvate–> acetyl CoA

A

B1, B2, B3, B5

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25
Q

Cori Cycle takes lactate from

A

mucle to liver to recreate glucose

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26
Q

Glucose to pyruvate is reducing NAD to

A

NADH

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27
Q

High pyruvate (2 nutrient cause)

A

Low b1 (can be depleted by alcohol) low b5

28
Q

Treatment for High Lactate

A

B1 in infants

29
Q

Treatment for high levels of Beta-hydroxybutryate

A

Chromium, vanadium, low carb diet

30
Q

Oxalate + _____ = kidney stones

A

calcium

31
Q

treatment for high oxalate

A

MG, citrate, vitamin D, antioxidants

32
Q

Central energy pathway markers (8)

A

citrate, isocitrate, cis aconitate, a-ketobutarate, succinate, fumarate, malate, hydroxymethylglutarate

33
Q

Acetyl-CoA—> citrate (enzyme)

A

citrate synthetase

34
Q

Citrat –> cis-Aconitate —> isocitrate (enzyme)

A

aconitase

35
Q

Citrat –> cis-Aconitate —> isocitrate (nutrients) 2

A

Fe, cysteine

36
Q

Isocitrate —> A-KG (enzyme)

A

isocitrate dehydrogenase

37
Q

Isocitrate —> A-KG (nutrients) 3

A

Mn, Mg, NAD

38
Q

Isocitrate —> oxalosuccinate –> A-KG (enzyme)

A

isocitrate dehydrogenase/decarboxylase

39
Q

Isocitrate —> oxalosuccinate –> A-KG (NUTRIENTS)

A

mn, mg, NADP

40
Q

A-KG —> succinyl-CoA (enzyme)

A

a-KG dehydrogenase complex

41
Q

A-KG—-> succinyl-CoA (vitamin)

A

TPP, lipoic acid, FAD, NAD, MG, trans-succinylase, CoA/B5)

42
Q

Succinyl-CoA—> succinate (enzyme)

A

Succinyl thiokinase

43
Q

succinate–>fumarate (enzyme)

A

succinate dehydrogenase

44
Q

Fumarate —-> malate (enzyme)

A

fumarase

45
Q

Malate—> oxaloacetate (enzyme)

A

malate dehydrogenase

46
Q

OAA+acetyl-CoA–> citrate =

A

starts cycle all over again

47
Q

ETC needs

A

CoQ10

48
Q

CItrate is high with low

A

ARG

49
Q

High ALL TCA cycle intermediates =

A

low CoQ10

50
Q

low citrate is correlated with

A

kidney stones

51
Q

Isocitrate dehydrogenase converts isocitrate–> A-KG requires

A

MG, mn, nAD

52
Q

High cis-aconatate with low isocitrate defect in

A

aconitase

53
Q

high cis-aconitate with low isocitrate requires (2)

A

Fe, cysteine

54
Q

deficiencines in what vitamins lead to accumulation of a-kg

A

B vitamins

55
Q

glutamate dehydrogenase (GDH) concerts glu->a-kg requiring which nutrient

A

B3

56
Q

treat high succinate with

A

CoQ10 and B2

57
Q

lleu and lle-> succinate requires what nutrient

A

b12

58
Q

high fumarate and malate

A

low Coq10

59
Q

high malate may be due to what nutrient deficinecy

A

b12

60
Q

what nutrients will refill fumarate

A

phe/tyr

61
Q

HMG-CoA lyase requires what nutrient

A

Mg and Mn

62
Q

deficiency of HMG CoA lyase leads to high

A

HMG

63
Q

BCKA degradation via BCKDC requires

A

B1 B2 b3 B5 and lipoic acid

64
Q

Treatment of IEM

A

protein resctricted diet and supplemnentation with carnitine and glycine

65
Q

deficiency of what vitamin leads to accumulation of xanthurenate and kynurenate via kynurenin pathway

A

b6

66
Q

Kynureniase requires

A

b6

67
Q

cofactor for carboxylases

A

biotin