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Biochemistry > Biochemistry 2/2, First Aid for the USMLE Step 1 > Flashcards

Biochemistry 2/2, First Aid for the USMLE Step 1 Flashcards

1
Q

Vitamin B1

A

Thiamine

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2
Q

Vitamin B2

A

Riboflavin

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3
Q

Vitamin B3

A

Niacin

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4
Q

Vitamin B5

A

Pantothenic acid

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5
Q

Vitamin B6

A

Pyridoxine

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6
Q

Vitamin B7

A

Biotin

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7
Q

Vitamin B9

A

Folate

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8
Q

Vitamin B12

A

Cobalamin

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9
Q

Vitamin that reguates Cl- channel in nerve conduction

A

B1

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10
Q

Vitamin essential for normal differentiation of epithelial cells into specialized tissue

A

Vitamin A

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11
Q

Vitamin A is found in what food stuff

A

Liver and leafy vegetables

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12
Q

Vitamin A prevents what cellular adaptation

A

Squamous metaplasia

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13
Q

Vitamin A is used to treat what CA

A

AML M3

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14
Q

Seen in conjunctiva of patients with Vitamin A deficiency

A

Bitot spots

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15
Q

T/F Vitamin A is teratogenic

A

T

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16
Q

Drug which is a vitamin A derivative that is contraindicated in pregnancy

A

Isotretinoin

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17
Q

Deficient in beriberi

A

B1

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18
Q

Deficient in Wernicke-Korsakoff syndrome

A

B1

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19
Q

Classic triad of Wernicke Korsakoff syndrome

A

1) Confusion 2) Ophthalmoplegia 3) Ataxia

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20
Q

Dry vs wet beriberi: Polyneuritis, symmetrical muscle wasting

A

Dry beriberi

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21
Q

Dry vs wet beriberi: High-output cardiac failure (dilated cardiomyopathy), edema

A

Wet beriberi

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22
Q

Vitamin component of FAD and FMN, used as cofactors in redox reactions

A

B2

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23
Q

ATP per B2

A

2

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24
Q

2 Cs of B2

A

Cheilosis and corneal vascularisation

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25
Q

Vitamin component of NAD and NADP, used as cofactors in redox reactions

A

B3

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26
Q

Vitamin B3 is derived from what amino acid

A

Tryptophan

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27
Q

ATP per B3

A

3

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28
Q

3 Ds of B3

A

Diarrhea, dementia, and dermatitis (C3/4 dermatome; broad collar rash; Casal necklace)

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29
Q

Metabolic disorder that can cause pellagra due to decreased niacin from tryptophan (decreased tryptophan absorption)

A

Hartnup disease

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30
Q

Facial flushing in niacin excess is due to

A

Prostaglandin release, NOT HISTAMINE!

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31
Q

How can facial flushing from niacin be avoided

A

Take aspirin with niacin

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32
Q

Vitamin that is an essential component of coenzyme A (CoA, a cofactor for acyl transfers) and fatty acid synthase

A

B5

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33
Q

Deficiency of this vitamin causes sideroblastic anemias due to impaired hemoglobin synthesis and iron excess

A

B6

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34
Q

Deficiency of this vitamin causes peripheral neuropathy

A

B6

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35
Q

Vitamin which is a cofactor for carboxylation enzymes (which add a 1-carbon group)

A

B8

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36
Q

Food stuff that avidly binds biotin

A

Egg whites (avidin; excessive ingestion of raw egg whites can cause deficiency of biotin)

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37
Q

Vitamin that is important for the synthesis of nitrogenous bases in DNA and RNA

A

B9

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38
Q

B9 is found in what foodstuff

A

Green leafy vegetables [FOLate from FOLiage]

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39
Q

B9 is absorbed in

A

Jejunum

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40
Q

Vitamin deficiencies that can cause macrocytic, megaloblastic anemia; hypersegmented polymorphonuclear cells (PMNs)

A

B9, B12

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41
Q

Symptom that distinguishes folate from B12 deficiency

A

No neurologic symptoms in folate defciency, present in B12

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42
Q

Vitamin that serves as cofactor for homocysteine methyltransferase (transfers CH3 groups) and methylmalonyl-CoA mutase

A

B12

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43
Q

Foodstuff that contain vitamin B12

A

Animal products

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44
Q

Vitamin which is an antioxidant and facilitates iron absorption

A

Vitamin C

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45
Q

Vitamin which is necessary for hydroxylation of proline and lysine in collagen synthesis

A

Vitamin C

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46
Q

Vitamin which is necessary for dopamine β-hydroxylase, which converts dopamine to norepinephrine

A

Vitamin C

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47
Q

Vitamin that can be used as ancillary treatment for methemoglobinemia by reducing iron to the ferrous form

A

Vitamin C

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48
Q

Corkscrew hair

A

Vitamin C deficiency

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49
Q

Form of vitamin D ingested from plants

A

D2/ergocalciferol

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50
Q

Form of vitamin D consumed in milk

A

D3/cholecalciferol

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51
Q

Vitamin D formed in sun-exposed skin

A

D3/cholecalciferol

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52
Q

Vitamin D is formed in what layer of the skin

A

Stratum basale

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53
Q

Storage form of vitamin D

A

25-OH D3

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54
Q

Active form of vitamin D

A

1,25-(OH)2 D3/calcitriol

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55
Q

Deficiency of vitamin D in children

A

Rickets

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56
Q

Deficiency of vitamin D in adults

A

Osteomalacia

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57
Q

Why is there vitamin D excess in granulomatous disease

A

Increased activation of vitamin D by epithelioid macrophages

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58
Q

Rickets, genu varum vs valgum

A

Varum

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59
Q

Vitamin that can enhance anticoagulant effects of warfarin

A

Vitamin E

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60
Q

Deficiency of this vitamin may have neurologic presentation that appear similar to vitamin B12 deficiency, but without megaloblastic anemia, hypersegmented neutrophils, or serum methylmalonic acid levels.

A

Vitamin E

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61
Q

Cofactor for the γ-carboxylation of glutamic acid residues on various proteins required for blood clotting

A

Vitamin K

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62
Q

Vitamin K dependent clotting factors

A

IX, X, VII, II, protein C, protein S

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63
Q

Vitamin K antagonist

A

Warfarin

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64
Q

T/F Vitamin K is supplied by breast milk

A

F

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65
Q

Mineral essential for the activity of 100+ enzymes

A

Zinc

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66
Q

Vitamin important in formation of transcription factor motif

A

Zinc (transcription factor motif is aka zinc fingers)

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67
Q

Deficiency of this vitamin results in delayed wound healing

A

Zinc

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68
Q

Acrodermatitis enteropathica is due to deficiency of what enzyme

A

Zinc

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69
Q

Vitamin deficiency that may predispose to alcoholic cirrhosis

A

Zinc

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70
Q

Inhibits alcohol dehydrogenase and is an antidote for methanol or ethylene glycol poisoning

A

Fomepizole

71
Q

Inhibits acetaldehyde dehydrogenase (acetaldehyde accumulates, contributing to hangover symptoms)

A

Disulfiram

72
Q

Limiting reagent in ethanol metabolism

A

NAD+

73
Q

Ethanol metabolism NADH/NAD+ ratio in liver, causing

A

1) Pyruvate > lactate (lactic acidosis) 2) Oxaloacetate > malate (prevents gluconeogenesis > fasting hypoglycemia) 3) Dihydroxyacetone phosphate > glycerol- 3-phosphate (combines with fatty acids to make triglycerides > hepatosteatosis)

74
Q

Pathways that take place in the mitochondria

A

1) Fatty acid oxidation (β-hydroxylase) 2) acetyl- CoA production 3) TCA cycle 4) oxidative phosphorylation 5) ketogenesis

75
Q

Pathways that take place in the cytoplasm

A

1) Glycolysis 2) fatty acid synthesis 3) HMP shunt 4) protein synthesis (RER) 5) steroid synthesis (SER) 6) cholesterol synthesis

76
Q

Enzyme deficient in PKU

A

Phenylalanine hydroxylase or THB4 cofactor (malignant PKU)

77
Q

T/F Screening for PKU should be done immediately after birth

A

F, 2-3 days after birth because baby is normal at birth due to maternal enzyme

78
Q

Disorder of aromatic amino acid metabolism

A

PKU

79
Q

What food stuff must patients with PKU avoid

A

Aspartame (artificial sweetener that contains phenylalanine)

80
Q

Enzyme deficient in albinism

A

Tyrosinase

81
Q

Enzyme deficient in alkaptonuria

A

Homogentisate oxidase

82
Q

Enzyme inhibited by Carbidopa in catecholamine synthesis

A

DOPA decarboxylase

83
Q

Enzyme stimulated by cortisol in catecholamine synthesis

A

Phenylethanolamine-N-methyltransferase

84
Q

Enzyme deficient in MSUD

A

α-ketoacid dehydrogenase

85
Q

Most abundant α-ketoacid in the blood of patients with MSUD

A

Leucine

86
Q

Supplemented in patients with MSUD

A

Thiamine

87
Q

Congenital deficiency of homogentisate oxidase in the degradative pathway of tyrosine to fumarate

A

Alkaptonuria

88
Q

Alkaptonuria is aka

A

Ochronosis

89
Q

Direction of lens subluxation in homocystinuria

A

Downward and inward

90
Q

Hereditary defect of renal PCT and intestinal amino acid transporter that prevents reabsorption of Cysteine, Ornithine, Lysine, and Arginine (COLA)

A

Cystinuria

91
Q

Test to diagnose cystinuria

A

Urinary cyanide-nitroprusside test

92
Q

Microscopic shape of cystine crystals

A

Hexagonal

93
Q

Treatment for cystinuria

A

1) Urinary alkalinization (potassium citrate or acetazolamide) 2) Chelating agents (penicillamine)

94
Q

Refers to the one to four residues remaining on a branch after glycogen phosphorylase has already shortened glycogen

A

Limit dextrin

95
Q

Debranching enzymes in glycogenolysis

A

1) 4-α-D-glucanotransferase 2) α-1,6-glucosidase

96
Q

Glycogen storage disease: Severe fasting hypoglycemia

A

Von Gierke

97
Q

Glycogen storage disease: Cardiomegaly, hypertrophic cardiomyopathy, exercise intolerance

A

Pompe

98
Q

Glycogen storage disease: Milder form of type I with normal blood lactate levels

A

Cori

99
Q

Glycogen storage disease: Increased glycogen in muscle, but muscle cannot break it down

A

McArdle

100
Q

Enzyme deficient: Von Gierke

A

Glucose-6-phosphatase

101
Q

Enzyme deficient: Pompe

A

Lysosomal α-1,4-glucosidase (acid maltase)

102
Q

Enzyme deficient: Cori

A

Debranching enzyme ( α-1,6-glucosidase)

103
Q

Enzyme deficient: McArdle

A

Skeletal muscle glycogen phosphorylase (myophosphorylase)

104
Q

Treatment for McArdle

A

Vitamin B6 (cofactor)

105
Q

MC sphingolipidosis

A

Gaucher

106
Q

Sphingolipidosis: Aseptic necrosis of femur

A

Gaucher

107
Q

Sphingolipidosis: Lysosomes with onion skin

A

Tay-Sachs

108
Q

Enzyme deficient and substance that accumulates: Fabry

A

α-galactosidase A; Ceramide trihexoside

109
Q

Enzyme deficient: Gaucher disease

A

Glucocerebrosidase (β-glucosidase); Glucocerebroside

110
Q

Enzyme deficient: Niemann-Pick disease

A

Sphingomyelinase; Sphingomyelin

111
Q

Enzyme deficient: Tay-Sachs disease

A

Hexosaminidase A; GM2 ganglioside

112
Q

Enzyme deficient: Krabbe disease

A

Galactocerebrosidase; Galactocerebroside, psychosine

113
Q

Enzyme deficient: Metachromatic leukodystrophy

A

Arylsulfatase A; Cerebroside sulfate

114
Q

Gargoylism

A

Hurler

115
Q

Mild Hurler + aggressive behavior

A

Hunter

116
Q

Corneal clouding

A

Hurler, NOT hunter

117
Q

Hurler vs hunter: X-linked recessive

A

Hunter

118
Q

Enzyme deficienct: Hurler

A

α-l-iduronidase

119
Q

Enzyme deficient: Hunter

A

Iduronate sulfatase

120
Q

Lysosomal storage diseases with increased incidence in Ashkenazi Jews

A

Tay-Sachs, Niemann-Pick, and some forms of Gaucher disease

121
Q

Long-chain fatty acid (LCFA) degradation requires

A

Carnitine-dependent transport into the mitochondrial matrix

122
Q

Inherited defect in transport of long chain fatty acids into the mitochondria toxic accumulation

A

Systemic 1° carnitine deficiency

123
Q

Autosomal recessive disorder of fatty acid oxidation characterized by decreased ability to break down fatty acids into acetyl-CoA > accumulation of 8- to 10-carbon fatty acyl carnitines in the blood and hypoketotic hypoglycemia

A

Medium-chain acyl-CoA dehydrogenase deficiency

124
Q

Shunts glucose and FFA toward the production of ketone bodies

A

Buildup of acetyl-CoA

125
Q

Breath smells like acetone (fruity odor)

A

Ketoacidosis

126
Q

Urine test for ketones does not detect

A

_-hydroxybutyrate

127
Q

1g protein = how many kcal

A

4

128
Q

1g CHO = how many kcal

A

4

129
Q

1g fat = how many kcal

A

9

130
Q

1g alcohol = how many kcal

A

7

131
Q

Glycogen reserves are depleted after how many hours of starvation

A

24/1 day

132
Q

Rate-limiting enzyme in cholesterol synthesis

A

HMG-CoA reductase

133
Q

Hormone that induces HMG-CoA reductase

A

Insulin

134
Q

HMG-CoA reductase converts HMG-CoA to

A

Mevalonate

135
Q

2⁄3 of plasma cholesterol esterified by ___

A

Lecithin-cholesterol acyltransferase (LCAT)

136
Q

Competitively and reversibly inhibit HMG-CoA reductase

A

Statins

137
Q

Enzyme: Degradation of dietary triglycerides (TGs) in small intestine

A

Pancreatic lipase

138
Q

Enzyme: Degradation of TGs circulating in chylomicrons and VLDLs

A

Lipoprotein lipase (LPL)

139
Q

Enzyme: Found on vascular endothelial surface; degrade circulating TGs

A

Lipoprotein lipase (LPL)

140
Q

Enzyme: Degradation of TGs remaining in IDL

A

Hepatic TG lipase (HL)

141
Q

Enzyme: Degradation of TGs stored in adipocytes

A

Hormone-sensitive lipase

142
Q

Enzyme: Catalyzes esterification of cholesterol

A

LCAT

143
Q

Mediates transfer of cholesterol esters to other lipoprotein particles

A

Cholesterol ester transfer protein (CETP)

144
Q

Apolipoprotein: Mediates remnant uptake

A

E

145
Q

Apolipoprotein: Activates LCAT

A

A-I

146
Q

Apolipoprotein: Lipoprotein lipase cofactor

A

C-II

147
Q

Apolipoprotein: Mediates chylomicron secretion

A

B-48

148
Q

Apolipoprotein: Binds LDL receptor

A

B-100

149
Q

Lipoprotein: Transports cholesterol from liver to tissues

A

LDL

150
Q

Lipoprotein: Transports cholesterol from periphery to liver

A

HDL

151
Q

Lipoprotein: Delivers dietary TGs to peripheral tissue

A

Chylomicron

152
Q

Lipoprotein: Delivers cholesterol to liver in the form of chylomicron remnants, which are mostly depleted of their TGs

A

Chylomicron

153
Q

Lipoprotein: Secreted by intestinal epithelial cells

A

Chylomicron

154
Q

Lipoprotein: Delivers hepatic TGs to peripheral tissue

A

VLDL

155
Q

Lipoprotein: Secreted by liver

A

VLDL

156
Q

Lipoprotein: Formed in the degradation of VLDL. Delivers TGs and cholesterol to liver.

A

IDL

157
Q

Lipoprotein: Formed by hepatic lipase modification of IDL in the peripheral tissue. Taken up by target cells via receptor-mediated endocytosis.

A

LDL

158
Q

Lipoprotein: Mediates reverse cholesterol transport

A

HDL

159
Q

Lipoprotein: Acts as a repository for apolipoproteins C and E (which are needed for chylomicron and VLDL metabolism).

A

HDL

160
Q

Lipoprotein: Secreted from both liver and intestine.

A

HDL

161
Q

Type I dyslpidemia

A

Hyperchylomicronemia

162
Q

Type IIa dyslipidemia

A

Familial hypercholesterolemia

163
Q

Type IV dyslipidemia

A

Hypertriglyceridemia

164
Q

Familial dyslipidemia: Lipoprotein lipase deficiency or altered apolipoprotein C-II.

A

Type I

165
Q

Familial dyslipidemia: Absent or defective LDL receptors.

A

Type IIa

166
Q

Familial dyslipidemia: Causes accelerated atherosclerosis (may have MI before age 20), tendon (Achilles) xanthomas, and corneal arcus

A

Type IIa

167
Q

Familial dyslipidemia: Hepatic overproduction of VLDL.

A

Type IV

168
Q

T/F In hyperchylomicronemia, there is an increased risk for atherosclerosis

A

F

169
Q

Triglyceride level in hypertriglyceridemia that can cause acute pancreatitis

A

> 1000 mg/dL

170
Q

Elevated in the blood: Hyperchylomicronemia

A

Chylomicrons, TG, cholesterol

171
Q

Elevated in the blood: Familial hypercholesterolemia

A

LDL, cholesterol

172
Q

Elevated in the blood: Hypertriglyceridemia

A

VLDL, TG

173
Q

Hydroxylation of proline and lysine takes place in the

A

RER

174
Q

Collagen has what amino acid in every 3rd position

A

Proline

Biochemistry (5 decks)

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