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Biochem Flashcards

1
Q

leukotriene targeted drugs

A

montelukast

zileutin

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2
Q

Rb

A

tumor suppressor

osteosarcoma, retinoblastoma

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3
Q

Niacin side effects

A

flushing of the face

can be aleviating by taking aspirin

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4
Q

Cystinuria

A

AR
defect in aa transporter for
cysteine, orthithine, lysine, arginine
recurrent stones

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5
Q

Chediak-Higashia syndrome mechanism

A

microtubule polymeraization defect leading to decreased phagocytosis

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6
Q

vincristine

A

Mphase specific

prevents microtubule polymeralization

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7
Q

Chediak HIgashi syndrome symptoms

A

recurrent pyogenic infections

partial albinism

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8
Q

vit c

A

proline hydroxylation in collagen synthesis

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9
Q

McArdle’s disease

A

AR
glycogen phosphorylase
increased glycogen in muscle that can’t be broken down
painful cramps, myoglobinuria

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10
Q

RNA polymerase I

A

makes rRNA

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11
Q

RNA polymerase II

A

makes mRNA

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12
Q

RNA polyermase III

A

makes tRNA

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13
Q

alpha-amanitin

A

inhibits RNA polyermase II

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14
Q

Kwashiorkor

A 
protein malnutrition (tryptophan and lysine)
Malnutrition, Edema, Anemia, Liver
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15
Q

21-hydroxylase def

A

decreased mc: hypotension, hyperkalemia

pseudoherm in females

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16
Q

17alpha-hydroxylase def

A

increased mc: hypertension, hypokalemia

males: ambiguous genitalia/undescended testes
females: normal, lack 2 sex characteristics

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17
Q

11b-hydroxylase def

A

decreased aldosterone, increased c: hypertension

masculinization

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18
Q

rate limiting enzyme of fatty acid synthesis

A

acetyl-CoA carboxylase

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19
Q

treatment for uterine leiomyomas

A

GnRH agonist/leuprolide

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20
Q

sympathetic alpha one

A

increases vascular smooth muscle contraction

Gq (phospholipase c)

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21
Q

phospholipase C

A

PIP2 –> Dag and IP3

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22
Q

DAG

A

protein kinase 3

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23
Q

IP3

A

Ca –> smooth muscle contraction

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24
Q

sympathetic alpha two

A

decreased sympathetic outflow

qi (adenylyl cylase)

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25
Q

adenylyl cyclase

A

Camp –> protein kinase a

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26
Q

protein kinase a

A

increased ca in heart

myoslin light chain kinase (smooth muscle)

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27
Q

sympathetic beta one

A 
increased heart rate, contractility
adenylyl cyclase (gs)
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28
Q

sympathetic beta two

A

vasodilation, bronchodilation
increases HR and contractility
Gs (adenylyl cyclase)

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29
Q

Niacin/B3 def

A

diarrhea, dermatitis, dementia

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30
Q

fatty liver in alcoholics

A

increased NADH:NAD ratio

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31
Q

first step of urea cycle

A

occurs in hepatocyte mitochondria

ornithine + carbamyl phosphate –> citrulline

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32
Q

Hartnup disease

A

AR
defective neutral amino acid transporter
tryptophan excretion in urine, can lead to pellagra

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33
Q

I cell disease

A

addition of mannose-6-phosphate to lysosome proteins

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34
Q

mannose-6-phosphate

A

add to traffick to lysosomes

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35
Q

nitroprusside

A

cyanide toxicity (cherry red lips)

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36
Q

mechanisms of cyanide toxicity

A

inhibit mitochondrial cytochrome oxidase

binds ferrous ion in hemoglobin

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37
Q

hereditary nonpolyposis colon cancer

A

mismatch repair defect

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38
Q

alpha ketoacid dehydrogenase def

A

maple syrup urine disease (AR)

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39
Q

homogentistic acid oxidase def

A

alkaptonuria (AR)

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40
Q

decreased phenylalanine hydroxylase

A

PKU (AR)

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41
Q

decreased tetrahydrobiopterin cofactor

A

PKU (AR)

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42
Q

cystathionine synthase def

A

homocystinuria (AR)

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43
Q

homocysteine methyltransferase def

A

homocystinuria (AR)

requires B12!

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44
Q

Maple Syrup Urine Disease

A

blocked degradation of branched AA (Ile, Leu, Val)

decreased alpha ketoacid dehydrogenase

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45
Q

electron carriers

A

NADH
NADPH
FADH2

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46
Q

acyl group carriers

A

coenzyme A

lipoamie

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47
Q

1 carbon unit carriers

A

tetrahydrofolates

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48
Q

aldehyde carriers

A

TPP

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49
Q

Ch3 carriers

A

SAM

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50
Q

Co2 carriers

A

Biotin

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51
Q

NAD+/NADP+ vitamin

A

B3

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52
Q

FAD+ vitamin

A

B2

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53
Q

NAD+ used in

A

catabolic processes

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54
Q

NADPH used in

A

anabolic processes
respiratory bust
p450
glutathione reducase

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55
Q

Li-Fraumeni syndrome

A

p53

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56
Q

Lesch-Nyhan syndrome

A 
def in purine salvage due to absence of HGPRT
results in excess uric acid production and de novo purine synthesis
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57
Q

Lesch-Nyhan syndrome transmission

A

x linked recessive

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58
Q

Lesch-Nyhan symptoms

A

retardation, self mutilation
hyperuricemia/gout
B12 def
choreoathetosis

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59
Q

donepezil

A

ache inhibitor (alzheimer’s treatment)

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60
Q

hypertriglyceridemia treatment

A

fibrates (increase activity of lipoprotein lipase)

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61
Q

hexokinase

A

high affinity/low Km
low Vmax
inhibited by g6p

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62
Q

glucose –> glucose 6 p

A

hexokinase or glucokinase

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63
Q

glucokinase

A

Liver and B cells of pancreas
low affinity/high Km
high Vmax
induced by insulin

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64
Q

SGLT location

A

enterocytes, nephrons

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65
Q

GLUT-1

A

insulin independent

RBCs, brain

66
Q

GLUT-2

A

bidirectional

B islet cells, liver, kidneys, small intestine

67
Q

GLUT-4

A

insulin dependent

adipose and skeletal tissue

68
Q

phosphorylation of mannose residues location

69
Q

renal gluconeogenesis

A

fructose-1,6,-biphosphatase is rate limiting

70
Q

Southern Blot

71
Q

Northern Blot

72
Q

cardiolipin

A

phospholipid on inner mitochondrial membrane

syphilis has Abs against

73
Q

rate limiting step of glycolysis

A

fructose 6 phosphate to fructose 1,6 bisphosphonate
PPFK
citrate inhibits PPFK

74
Q

homocystinuria vitamin link

A

B6 as cofactor

75
Q

porphobilinogen deaminase def

A

acute intermittent porphyria

76
Q

Wernicke’s Aphasia

A

located in left superior and middle temporal lobe

speak but make no sense

77
Q

endothelial cells normally have

A

NO, prostacyclin to prevent plaque formation

78
Q

S phase of cell cycle

A

DNA is synthesized

79
Q

PCOS treatment

A

spirolactone

80
Q

accumulation of cerebroside sulfate

A

metachromatic leukodystrophy/def of airsulfatase A

81
Q

urinary reducing substances

A

galactosemia

82
Q

symptoms of maple syrup urine disease

A

hypoglycemia

CNS defects, death

83
Q

V2 Receptor

A

Gs (adenylate cyclase)

84
Q

ACEI and g proteins

A

decreases Gq activity

85
Q

location of rxns of glycogenesis and glycogenolysis

86
Q

Beta blockers not given with cocaine use

A

unopposed alpha effects

87
Q

paclitaxel

A

m phase, disrupts mitotic spindle

88
Q

Hemoglobin left shift

A

decreased H, 2,3DPB, temp

89
Q

Radiation

A

DNA ds breaks

formation of free radicals

90
Q

telomerase

A

RNA dep DNA polymerase

91
Q

TTAGGG

A

added by telomerase

92
Q

hypoglycemia, hypoketonia

A

MCAD def

acyl-coa DH

93
Q

Vitamin A toxicity

A

skin changes

hepatic injury

94
Q

Essential fructosuria

A

asymptomatic, AR
defect in fructokinase
fructose appears in blood and urine

95
Q

Biotin required for

A

pyruvate, acetyl coA, propionyl, and betamethylcrotonyl coa CARBOXYLASE

96
Q

acid alpha glucosidase

A

Pompe’s disease (lysosomal)

97
Q

pantothenic acid

A

coenzyme A

98
Q

TCA cycle first step

A

coenzyme A + oxaloacetate –> citrate (citrate synthase)

99
Q

HMP shunt

A

source of NADPH from glucose-6-phosphate

no ATP used

100
Q

locations where HMP shunt happens

A

lactating mamary glands
liver
adrenal cortex
RBCs

101
Q

endothelial dependent vasodilation

A

NO from arginine

102
Q

Xeroderma pigmntous

A

defect in UV endonuclease

103
Q

stop codons

104
Q

aldolase B

A

fructose intolerance

decrease intake of both fructose and sucrose

105
Q

rRNA synthesis location

106
Q

galactoyl B-14 glucose

107
Q

transfer tRNA

A

3’ CCA tail

thymidine

108
Q

TNF alpha activates

A

serine kinases

109
Q

treatment of measles

110
Q

vit def and cardiac symptoms

A

beriberi/vitamin B1

111
Q

PRPP

A

accumulates in leesch-nyhan

112
Q

homeobox

A

transcription regulators

113
Q

thyroid hormone - type of receptors

114
Q

myc

A

transcription factors

115
Q

long fatty acid and amino acid synthesis location

A

peroxisomes

116
Q

orotic aciduria

A

defect in UMP synthase/can’t convert orotic acid to UMP

megoblastic anemia

117
Q

desmin stain

118
Q

vimentin stain

A

connective tissue

119
Q

cytokeratin stain

A

epithelial cells

120
Q

GFAP stain

121
Q

cardiac glycosides

A

inhibit Na/K ATPase

122
Q

type I collagen

A

bone, skin, tendon

123
Q

type II collagen

124
Q

type III collagen

125
Q

type IV collagen

A

basement membrane or basal lamina

126
Q

prader willi syndrome

A

paternal allelle not expressed

hyperphagia, obesity

127
Q

angelman’s syndrome

A

materinal allele not expressed

inappropriate laughter

128
Q

mitochondrial myopathies

A

myopathy and CNS disease

ragged red fibers

129
Q

PKD1

A

chromosome 16

130
Q

APC gene

A

chromosome 5 ( 5 letters in polyp)

131
Q

NF1

132
Q

NF2

133
Q

CFTR gene

134
Q

Cri-du-chat

A

microdeletion of short arm of chromosome 5

VSD

135
Q

fissures at corner of mouth

A

Vit b2/riboflavin

136
Q

niacin derived from

A

tryptophan

137
Q

biotin def

A

excess ingestion of raw eggs (avidin)

138
Q

vitamin b9

A

folic acid

139
Q

SAM composed of

A

ATP and methionine

depends on vitb12 and folate

140
Q

homocysteine converted into

A

methionine

141
Q

dopamine B hydroxylase

A

dopamine –> NE

need vitamin c for rxn

142
Q

D3

A

cholecalciferol

milk, sun exposed skin

143
Q

alcohol DH

A

cytosol

ethanol –> acetaldehdyge (requireds NAD)

144
Q

acetaldehyde DH

A

mitochondria

acetaldehyde –> acetate (requires NAD)

145
Q

fomepizole

A

inhibits alcohol DH

146
Q

disulfiram

A

inhibits acetaldehyde DH

147
Q

marasmus

A

muscle wasting

148
Q

what happens in cytoplasma

A

glycolysis
fatty acid synthesis
HMP shunt, cholesterol synthesis
protein synthesis -RER, steroid synthesis on SER

149
Q

what happens both in mitochondria and cytoplasm

A

HUG

heme synthesis, urea cycle, gluconeogensis

150
Q

glyclosis rate limiting enzyme

151
Q

gluconeogensis rate limiting enzyme

A

fructose 1,6 bisphosphatase

152
Q

TCA cycle rate limiting enzyme

A

isocitrate DH

153
Q

glycogen synthesis RLE

A

glycogen synthase

154
Q

glyocenolysis RLE

A

glycogen phosphorylase

155
Q

HMP shunt RLE

156
Q

de novo pyrimidine synthesis RLE

A

carbamoyl phosphate synthetase II

157
Q

de novo purine synthesis RLE

A

glutamine-PRPP amidotransferase

158
Q

urea cycle RLE

A

carbamoyl phosphate sythetase I

159
Q

fatty acid synthesis RLE

A

acetyl coa carboxylase

160
Q

fatty acid oxidation RLE

A

carnitine acyltransferase I

161
Q

ketogensosi RLE

A

hmg-coa synthase

162
Q

cholesterol synthesis RLE

A

hmg-coa reducatase

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