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USMLE Rapid Reviews > Biochemistry > Flashcards

Biochemistry Flashcards

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USMLE® Step 1 flashcards
1
Q

Glycolysis

A

Phosphofruktokinase-1 (PFK-1)

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2
Q

Gluconeogenesis

A

Fructose-1,6-bisphosphate

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3
Q

TCA cycle

A

Isocitrate Dehydrogenase

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4
Q

Glycogen synthesis

A

Glycogen synthase

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5
Q

Glycogenolysis

A

Glycogen phosphorylase

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6
Q

HMP shunt

A

Glucose 6 phosphate dehydrogenase (G6PD)

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7
Q

De novo pyrimidine synthesis

A

Carbamoyl phosphate synthetase II

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8
Q

De novo purine synthesis

A

Glutamine PRPP amidotransferase

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9
Q

Urea Cycle

A

Carbamoyl phosphate synthetase I

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10
Q

Fatty Acid Oxidation

A

Carnitine acyltransferase I

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11
Q

Fatty acid synthesis

A

Acetyl CoA carboxylase

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12
Q

Ketogenesis

A

HMG-CoA synthase

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13
Q

Cholesterol Synthesis

A

HMG-CoA reductase

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14
Q

glucose to glucose-6-phosphate

A 
Hexokinase
High affinity (low Km), uninduced by insulin
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15
Q

glucose to glucose-6-phosphate in liver and B-cells of pancrease

A

Glucokinase
low affinity (high Km), induced by insulin
phophorylates excess glucose

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16
Q

Carries amino acids to liver from muscle

A

alanine

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17
Q

Location of TCA

A

mitochondria

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18
Q

NADPH

A 
Reduces Glutathione
def = hemolytic anemia, heinz bodies, bite cells
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19
Q

Fructose intolerance:

hypoglycemia, jaundice, cirrhosis, vomiting

A

def of Aldolase B

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20
Q

Amino Acids required during growth

A

Arg and His.

Are increased in histones, which bind neg charged DNA

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21
Q

Hyperammonemia: acquired or urea cycle enzyme def

A

Results in excess NH4, which depletes alpha-ketoglutarate, leading to inhibition of TCA

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22
Q

MC urea disorder. Excess carbamoyl phosphate is converted to orotic acid.

A

Ornithine transcarbamoylase deficiency.

results in hyperammonemia

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23
Q

Phenylalanine derivatives

A

–>Tyrosine –> Dopa –> dopamine –> NE –>Epi

melanin comes from Dopa

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24
Q

Tryptophan derivatives

A

B6 –> Niacin –> NAD/NADP
or
BH4 –> serotonin –> melatonin

25
Q

Glutamate derivatives

A

B6 –> GABA

26
Q

Mental retardation, growth retardation, sezures, fair skin, musty odor, infant

A 
Phenylketonuria
def phenylalanine hydroxylase
27
Q

dark connective tissue and urine turns black on prolonged exposure to light

A 
Alkaptonuria
def of homogentisic acid oxidase
28
Q

Albanism

A

deficiency of tyrosine, defective tyrosine transporters, or lack of migration of neural crest cells.

29
Q

Inc homosystein in urine, mental retardation, tall, lens sublexation, athrosclerosis

A

Homocystinuria

  1. cystathione synthase deficiency
  2. def B6
  3. Homocysteine methyltransferase (req B12)
30
Q

Cystinuria

A

Staghorn calculi

31
Q

Maple syrup urine

A 
def of alpha-ketoacid dehydrogenase.
blocks degradation of branched amino acids
32
Q

tryptophan excreted in urine and decreased absorption in gut

A

Hartnups. Leads to pellagra

33
Q

Von Gierkes disease

A

Glucose-6-phosphate def

34
Q

Pompe’s disease

A

Lysosomal alpha 1,4 glucosidase

35
Q

Cori’s Disease

A

Debranching enzyme (alpah-1,6-glucosidase

36
Q

McArdles:

painful muscle cramps, myoglobinuria with strenuous exercise.

A

glycogen phosphorylase def in skeletal muscle

37
Q

peripheral neuropathy of hands and feet, angiokeratomas, cardio/renal disease

A

Fabry’s: a-galctosidase A def

38
Q

Hepatosplenomegaly, crumpled up tissue cells

A

Gaucer’s disease: Glucocerbrosidase def

39
Q

cherry red spots on macula, neurodegeneraion, hepatosleenomegally

A

Niemann-picks disease: sphingomyelinase def

40
Q

cherry red spots on macula, neurodegeneraion, No hepapatospleenomegaly

A

Tay-sachs: Hexosaminidase A

41
Q

Peripheral neruopathy with optic atrophy, developmental delay

A

Krabbe’s disease: B-galactocerebrosidase

42
Q

Central and perish demylination with ataxia and dementia

A

Metachromatic leukodystrophy: arylsulfatase A def

43
Q

Developmental delay, gargoylism, airway obstruction, corneal clouding

A

Hurler’s: a-L-iduronidase

Accumulation of: Heparn and dermatan sulfate

44
Q

agressive behavior, no corneal clouding, mild hurlers

A

Hunter’s: Iduronate sulfatase def.
accumulation: Heparan and Dermatan sulfate.
XR

45
Q

Cholesterol Synthesis

A

HMG-CoA reductase

“-statins” inhibit

46
Q

Mediates remnant uptake

A

Apo-E

47
Q

Mediates cylomicron secretion

A

Apo-B48

48
Q

Binds LDL receptor

A

Apo-B100

49
Q

Transports cholesterol from liver to tissue

A

LDL

50
Q

Transports cholesterol from periphery to liver

A

HDL

51
Q

Delivers dietary TG to peripheral tissue. Delivers cholesterol to liver.

A

Chylomicron

52
Q

Delivers hepatic TG to peripheral tissue. Secreted by liver.

A

VLDL

53
Q

Formed tin the degredation of VLDL. Delivers TG and cholesterol to liver.

A

IDL

54
Q

Delivers hepatic cholesterol to peripheral tissue. formed by lipoprotein lipase

A

LDL

55
Q

Acts as a repository for Apo E and Apo C (which are needed for chylomicron and VLDL metabolism)

A

HDL

56
Q

Increased chylomicrons in blood, pancreatitis, hepatomegaly, pruitic xanthomas

A

hyper-chylomicronemia.

Lipioprotein lipase deficiency or altered Apo C2.

57
Q

accelerated atherosclerosis, tendon xanthomas, and corneal arcus

A

Familial hypercholestrolemia.

LDL receptor def

58
Q

infant. failure to thrive, steatorrhea, ancanthocytosis, ataxia, night blindness

A

Abetalipoproteinemia. inability to synthasize lipoproteins due to def in aboB100 or apoB48

USMLE Rapid Reviews (6 decks)

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