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Spring 2015 > Block 3 Biochem > Flashcards

Block 3 Biochem Flashcards

1
Q

what is the committed reaction in steroid hormone sythesis?

A

cholesterol to pregnenolone

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2
Q

3 hydroxylations of progesterone makes

A

cortisol

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3
Q

2 hydroxylations and an oxidation of progesterone makes

A

aldosterone

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4
Q

3 beta hydroxysteroid dehyd. deficiency results in

A

pregnenolone buildup, marked salt excretion. also female genitalia

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5
Q

17 alpha hydroxylase deficiency results in

A

no sex hormones or cortisol. more aldosterone made

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6
Q

21 alpha hydroxylase deficiency-

A

no gluco or mineralocorticoids made

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7
Q

Angiotensin activates what protein kinase to stimulate aldosterone?

A

PKC

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8
Q

pepsinogen is secreted by _____

A

chief cells

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9
Q

pepsin is a ______ protease

A

aspartate

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10
Q

HCl is secreted by _______

A

parietal cells

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11
Q

signals the release of bicarb from the pancreas-

A

secretin

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12
Q

what converts trypsinogen to trypsin?

A

enteropeptidase

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13
Q

what activates most pancreatic zymogens?

A

trypsin

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14
Q

serine proteases are what kind of peptidases?

A

endopeptidases

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15
Q

trypsin cuts at what AA’s

A

C terminus arginines and lysines

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16
Q

elastase cuts what kinds of AA’s

A

small ones (ala, gly, ser)

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17
Q

are metallopeptidases endo or exo peptidases?

A

exo

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18
Q

Defect in COAL transport can lead to

A

cystinuria

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19
Q

hartnup disorder-

A

inability to reabsorb bulky AA’s

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20
Q

what coenzyme do aminotransferases use?

A

PLP (B6) derivative

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21
Q

which 2 aa’s do not undergo aminotransferase rxns

A

lysine and threonine

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22
Q

where is DAO located?

A

in peroxisomes

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23
Q

Cahill cycle-

A

alanine from muscle is transported to liver to be converted to pyruvate

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24
Q

blood sugar conversion factor. 1 mM = __ mg/dL

A

18

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25
Q

list the 4 metabolic enzymes that are active while phosphorylated

A

hormone sensitive lipase, glycogen phosphorylase kinase, and glycogen phosphorylase, fructose bisphosphate phosphatase 2

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26
Q

binge drinking can drive OAA towards malate production, leading to

A

alcoholic ketoacidosis, as there is now excess acetyl CoA

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27
Q

after 3 weeks of fasting, muscle quits using ketone bodies for energy, only using FFA. This makes it easier for….

A

brain to use ketone bodies

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28
Q

during prolonged fasting, what provides 50% of glucose to muscle?

A

kidneys

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29
Q

which secretion of adipose tissue improves insulin sensitivity?

A

adiponectin

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30
Q

as we get fatter, what happens to adiponectin levels?

A

they decrease

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31
Q

how do FFA and TNF alpha promote insulin resistance?

A

they bind to serine and threonine residues on insulin receptors and make the tyrosine residues unavailable to do its thing

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32
Q

macrovascular complications begin with

A

hyperinsulinemia

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33
Q

microvascular complications begin with

A

impaired glucose tolerance

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34
Q

BMI =

A

weight in kg/height m^2 or weight lb/height^2 inches x 703

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35
Q

do non-diabetic obese people have hyperglycemia?

A

no

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36
Q

what inhibits the synthesis of THF in bacteria?

A

sulfonamide

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37
Q

Methotrexate:

A

anti cancer drug. stops all synthesis of THF

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38
Q

what enzyme does methotrexate target?

A

dihydrofolate reductase

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39
Q

how is excess folate a problem?

A

can mask B12 deficiency

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40
Q

B12 aka

A

cobalamin

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41
Q

B12 used for synthesis of

A

methionine

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42
Q

enzyme responsible for uptake of B12

A

intrinsic factor

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43
Q

1 kg = ___ lbs

A

2.2

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44
Q

ideally our calories should come from (%)
carbs
fats
protein

A

carbs 55%
fats 25%
protein 20%

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45
Q

men should get ___ g fiber

A

38

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46
Q

essential n-3 FA in humans

A

alpha linoleic acid

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47
Q

which vitamins prevent CHD and how?

A

B6,12, and 9. Lower homocysteine levels

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48
Q

saturated fatty acids ___ LDL and ___ HDL

A

increase

no change

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49
Q

n-6 PUFA’s ____ LDL and ____ HDL

A

decrease both

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50
Q

n-3 effect on LDL/HDL

A

none

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51
Q

B12 is used for _______ catabolism, in which it is a coenzyme for ________

A

odd chain fatty acid\

methylmalonyl CoA mutase

52
Q

deficiency of B12 =

A

pernicious anemia

53
Q

B6 aka

A

pyridoxal phosphate

54
Q

B6 is a cofactor for sythesis of:

A

heme and histamine

55
Q

Beriberi disease associated with what vitamin?

A

Thiamine

56
Q

wericke-korsakoff syndrome associated with what vitamin?

A

Thiamine

57
Q

niacin is a precursor of

A

NADH and NADPH

58
Q

Niacin deficiency =

A

pellagra

59
Q

niacin inhibits _____ in adipose tissue

A

lipolysis

60
Q

B2 aka

A

riboflavin

61
Q

B2/riboflavin is a precursor for

A

FAD and FMN

62
Q

Biotin is a coenzyme of _____ reactions

A

carboxylation

63
Q

B5 aka

A

pantothenic acid

64
Q

rhodopsin =

A

retinal + opsin

65
Q

defect in cleavage of glycine. diet restrictions ineffective

A

nonketotic hyperglycinemia

66
Q

cystathione synthase defect or B6 deficiency leading to accumulation of methionine. OR… a folic acid or B12 deficit, which would actually cause low levels of methionine

A

homocystinuria (looks like marfans)

67
Q

most common urea cycle disorder

A

ornithine transcarbamoylase deficiency

68
Q

ornithine transcarbamoylase deficiency would manifest as

A

respiratory alkalosis, hyperammonemia, elevated orotic acid

69
Q

3 organic acidemias

A

propionic, methylmalonic and glutaric acidemia

70
Q

organic acidemia-

A

defect leading to accumulation of intermediates of catabolism of macromolecules.

71
Q

Pt has wide anion gap, ketonuria, and maybe hyperammonemia. what kind of disease?

A

organic acidemia

72
Q

propionic acidemia-

A

biotin deficiency or enzyme deficient, accumulation of propionyl coa

73
Q

methylmalonic acidemia-

A

enzyme or B12 deficiency, accumulation ofmethylmalonyl CoA

74
Q

Hypoketotic/hypoglycemic patient has no ketones in urine. What do they have?

A

Medium chain Acyl CoA dehydrogenase defect

75
Q

patient has hypoglycemia, low levels of ketones, and metabolic acidosis. What do they have?

A

Long chain acyl CoA dehydrogenase deficiency

76
Q

cofactor for tyrosine hydroxylase

A

BH4

77
Q

rate limiting step of catecholamine sythesis

A

tyrosine hydroxylase making DOPA

78
Q

DOPA decarboxylase cofactor

A

PLP

79
Q

dopamine–> norepi enzyme

A

dopamine beta hydroxylase

80
Q

Norepi–> epi enzyme

A

phenylethanolamine-N-methyl-transferase

81
Q

rate limiting step of 5-HT synthesis-

A

tryptophan–>5 hydroxytryptophan

82
Q

rate limiting step of creatine synthesis

A

arginine+glycine–>guanidinoacetate+ornithine via amidinotransferase

83
Q

H2 histamine receptor stimulates-

A

gastric secretions, smooth muscle relaxation

84
Q

hereditary fructose intolerance ED

A

Aldolase B

85
Q

Von Gierke Type 1 ED

A

G6 phosphatase

86
Q

Von Gierke Type 2 ED

A

G6 translocase

87
Q

Pompe disease ED

A

lysosomal alpha glucosidase

88
Q

Cori disease ED

A

debranching enz.

89
Q

McArdle disease ED

A

muscle glycogen phosphorylase

90
Q

5 mucopolysaccharidoses-

A

hurler, hunter, sanfilipo, morquio, and maroteaux-lamy

91
Q

hurler syndrome ED

A

alpha L iduronase

92
Q

Hunter syndrome ED

A

iduronate sulfatase

93
Q

sanfilipo syndrome ED

A

heparan sulfate degrading enzyme def.

94
Q

Morquio synd. ED

A

keratin sulfate degrading enzyme def.

95
Q

Maroteaux-Lamy ED

A

arylsulfatase B

96
Q

Neimann Pick ED

A

sphingomyelinase

97
Q

Tay Sachs ED

A

Hexosaminidase A

98
Q

GM1 ganglioacidosis ED

A

beta galactosidase

99
Q

Metachromatic leukodystrophy ED

A

arylsulfatase A

100
Q

Alpha-mannosidosis ED

A

alpha D mannosidase

101
Q

zellweger syndrome-

A

cant break down very long chain fatty acids

102
Q

Smith-Lemli-Opitz syndrome-

A

deficient in 7 dehydrocholesterol reductase. low cholesterol in body. polydactyly

103
Q

cherry red macula associated with

A

lysosomal storage diseases (glycolipidoses)

104
Q

list the nonessential glucogenic amino acids

A

All the A’s and G’s. + CSHP

105
Q

list the essential glucogenic AA’s

A

HMTV (Hate MTV)

106
Q

list the nonessential amino acid that is both gluco and ketogenic

A

Tyrosine

107
Q

list the 3 essential gluco/ketogenic aa’s

A

isoleucine, phenylalanine, and tryptophan (WIF)

108
Q

list the 2 essential ketogenic aa’s

A

Leucine, lysine (The L’s)

109
Q

are there nonessential ketogenic aa’s

A

NO!

110
Q

pro–>glu

A

proline oxidase

111
Q

arg–> ornithine

A

arginase

112
Q

OAA is synth. from what aa?

A

aspartate

113
Q

pyruvate is synth from what aa?

A

alanine

114
Q

ornithine–> glu

A

ornithine aminotransferase

115
Q

his–>glu

A

histidase

116
Q

ser–>pyruvate

A

serine dehydratase

117
Q

sery–>gly

A

serine hydroxymethyl transferase

118
Q

Thr–>succ CoA

A

threonine dehydratase

119
Q

Phe–>fumarate

A

phenylalanine hydroxylase (uses BH4)

120
Q

homocysteine–>cystathinone–>cysteine

A

cystathione beta synthase (req. B6)

121
Q

proline is formed by cyclization of

A

glutamine

122
Q

urocanic aciduria caused by deficiency in urocanase or

A

overactive histidase

123
Q

_____ deficiency mimics PKU

A

BH4

124
Q

homocystinuria ED

A

cystathione b sythase

125
Q

Alkaptonuria ED

A

homogenistic acid oxidase

126
Q

treatment for alkaptonuria-

A

inhibition of 4-hydroxyphenylpyruvate dioxygenase

Spring 2015 (9 decks)

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