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USMLE 2019 > Biochem > Flashcards

Biochem Flashcards

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USMLE® Step 1 flashcards
1
Q

rate-limiting step of bile synthesis

A

7a hydroxylase

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2
Q

rate-limiting step of heme synthesis

A

ALA synthase

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3
Q

enzyme that converts glucose to sorbitol and galactose to galactitol

A

Aldose reductase

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4
Q

enzyme that is deficient in severe combined immunodeficiency

converts adenosine to uric acid

A

Adenosine deaminase

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5
Q

rate-limiting step in cortisol synthesis

A

desmolase /
side chain cleavage enzyme
(cholesterol to pregnenolone)

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6
Q

familial cases of ALS are associated with what type of mutation

A

zinc-copper superoxide dismutase mutation

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7
Q

Lesch - Nyhan syndrome is characterized by deficiency of

A

HGPRT

defective purine salvage

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8
Q

enzyme affected in acute intermittent porphyria

A

porphobilinogen deaminase

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9
Q

amino acid derivatives of purine

A

Glutamine
Aspartate
Glycine

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10
Q

AA derivatives of pyrimidine

A

Aspartate

Glutamine

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11
Q

AA derivatives of heme

A

Glycine

Succinyl CoA

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12
Q

AA derivatives of urea

A

Arginine

Aspartate

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13
Q

rate limiting step in catecholamine synthesis

A

tyrosine hydroxylase

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14
Q

enzyme/s affected in lead poisoning

A

ferrochelatase

ALA dehydratase

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15
Q

enzyme deficient in albinism

A

tyrosinase

DOPA to melanin

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16
Q

rate-limiting enzyme of fatty acid oxidation

A

carnitine acyltransferase 1

mitochondrial enzyme

17
Q

vitamin B1 is a cofactor for which enzymes (4)

A

alpha ketoglutarate dehydrogenase
transketolase
pyruvate dehydrogenase
branched chain ketoacid dehydrogenase

18
Q

congenital deficiency of homogentisate oxidase

urine turns black
pigmented osteoarthritis

A

alkaptonuria

19
Q

congenital deficiency of homogentisate oxidase

urine turns black
pigmented osteoarthritis

A

alkaptonuria

USMLE 2019 (10 decks)

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