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biochemie USMLE Flashcards

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1
Q

Carried out in cytoplasm

A

Glycolisis, HMP shunt, fatty acid and steroid synthesis

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2
Q

Carried out in mitochondria

A

b-oxidation, acetyl-Co A production, Krebs cycle, oxidative phosphorylation

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3
Q

De novo pyrimidine synthesis

A

ATCase

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4
Q

Carried out in cytoplasm and mitochondria

A

Heme synthesis, Urea cycle, Gluconeogenesis

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5
Q

De novo purine synthesis

A

Glutamine-PRPP amidotransferasa

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6
Q

Glycolysis

A

PFK-1

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7
Q

Gluconeogenesis

A

F 1,6 BP

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8
Q

TCA cycle

A

Isocitrate DH

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9
Q

Glycogen synthesis

A

Glycogen synthasa

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10
Q

Glycogenolysis

A

Glycogen phosphorylase

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11
Q

HMP shunt

A

G6PDH

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12
Q

Fatty acid S.

A

ACC

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13
Q

Fatty acid O.

A

Carnitine acyltranferase I

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14
Q

Ketogenesis

A

HMG-Co A synthase

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15
Q

Cholesterol synthesis

A

HMG-Co A reductase

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16
Q

Heme S.

A

ALA synthase

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17
Q

Urea cycle

A

Carbamoyl phosphate synthase

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18
Q

Aerobic metabolism of glucose

A

30-32 ATP

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19
Q

Anaerobic glycolysis

A

2 ATP

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20
Q

CO2 carrier

A

Biotin

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21
Q

l-carbon units carrier

A

THF

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22
Q

Aldehydes

A

TPP

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23
Q

Uses of NADPH

A

Anabolic processes, respiratory burst, P-450

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24
Q

Hexokinase

A

Ubiquitous, high affinity, low capacity, uninduced by insulin, feedback by G-6-P

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25
Q

F 2,6 BP

A

Most potent activator of PFK

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26
Q

Glycolitic enzime deficiency

A

Hemolytic anemia (↓ Na K ATPasa activity)

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27
Q

Cofactors of Pyruvate and a-ketoglutarate DH complex

A

B1, B2, B3, B5 and lipoic acid (inhibited by arsenic)

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28
Q

Ketogenic aa

A

Lysine Leucine

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29
Q

Cory cycle sites

A

Liver → Muscle/RBCs

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30
Q

Cory cycle

A

Hepatic gluconeogenesis of lactate

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31
Q

ATP asa inhibitors

A

Oligomycin

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32
Q

Uncoupling agents

A

2,4 DNP, aspirin, termogenin

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33
Q

e- transport inhibitors

A

Rotenone, CN-, antimycin A, CO

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34
Q

Gluconeogenesis, irreversible enzimes

A

Pyruvate carboxylase, PEP carboxykinase, F 1,6 BP, G 6 P

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35
Q

HMP shunt product

A

NADPH

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36
Q

Fructose intolerance-symptoms

A

Hypoglicemia, jaundice, cirrhosis, vomiting

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37
Q

G 6 P DH deficiency-symptoms

A

Hemolytic anemia, heinz bodies, X-linked recessive

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38
Q

Aldolase B deficiency

A

Fructose intolerance

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39
Q

Essential fructosuria-

A

Defect in fructokinase

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40
Q

Galactosemia-symptoms

A

Cataracts, hepatosplenomegaly, mental retardation

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41
Q

Galactosemia

A

Abscence of Galactose-1-phosphate uridyltranferase

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42
Q

Lactase deficiency-symptoms

A

Bloating, cramps, osmotic diarrhea

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43
Q

Glucogenic/Ketogenic aa

A

Phe, Thr, Trp, ILe

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44
Q

Glucoegnic aa

A

Va, Met, Arg, His

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45
Q

Ammonia intoxication-symptoms

A

Tremor, slurred speech, somnolence, vomiting, cerebral edema, blurred vision

46
Q

Ammonia intoxication-Treatment

A

Benzoate or phenylbutirate

47
Q

Urea cycle, aa related

A

Aspartate, arginine

48
Q

Phenylalanine derivatives

A

Tyrosine (thyroxine), DOPA (melanin), dopamine, NE, epi

49
Q

Tryptophan derivatives

A

Niacin (NAD/NADP), serotonin-melatonin

50
Q

Histidine derivatives

A

Histamine

51
Q

Glycine derivatives

A

Porphyrin-Heme

52
Q

Arginine derivatives

A

Creatine, urea, NO

53
Q

Glutamate derivatives

A

GABA, glutathione

54
Q

Phenylketonuria, symptoms

A

Autosomal recessive. Mental and growth retardation, fair skin, eczema, musty body odor

55
Q

Phenylketonuria, defects

A

↓ phenylalanine hydroxilase or ↓tetrahidrobiopterin cofactor

56
Q

Tyrosine food sources

A

Meat, fish, milk, wheat, oats, leafy vegetables,bananas, nuts

57
Q

Alkaptonuria, defect

A

Homogentisic acid oxidase deficiency

58
Q

Alkaptonuria, symptoms

A

Arthralgias, dark connective tisue, urine to turn black on standing, bening disease

59
Q

Albinism, defects

A

Deficiency of tyrosinase or its transporters

60
Q

Homocystinuria, signs

A

Autosomal recessive. Mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation, atherosclerosis

61
Q

Homocystinuria, deficiencies

A

Cystathion synthase or homocysteine methyltranferase. ↓ affinity of cystathion synthase for pyridoxal phosphate)

62
Q

Cystinuria, defect

A

Transporter defect of Cys Orn Lys Arg

63
Q

Staghorn calculi

A

Cystinuria (AR)

64
Q

Maple syrup urine disease (AR)

A

Blocked degradation of branched aa due to ↓ a-ketoacid DH

65
Q

Branched aa

A

Ile Leu Val

66
Q

Maple syrup urine disease

A

CNS defects, mental retardation, urine smells like maple syrup

67
Q

Purine salvatage deficiencies

A

ADA deficiency, Lesh-Nyhan sd.

68
Q

ADA deficiency

A

Can cause SCID

69
Q

Lesh-Nyhan sd.

A

Absence of HGPRT (converts hipoxanthine→IMP and guanine→GMP)

70
Q

Lesh-Nyhan sd.

A

Retardation, self mutilation, aggression, hyperuricemia, gout, choreoathetosis

71
Q

100-meter sprint

A

Stored ATP, creatine phosphate, anaerobic glycolysis

72
Q

100-meter run

A

Stored ATP, creatine phosphate, anaerobic glycolysis, oxidative phosphorylation

73
Q

Marathon

A

Glycogen, FFA oxidation. Glucose for final sprinting

74
Q

Starvation after day 3

A

Hepatic formation of ketone bodies

75
Q

GLUT 2 receptor

A

b cells, liver, kidney

76
Q

GLUT 4 receptor (insuline responsive)

A

muscle and fat

77
Q

Proinsulin

A

insulin + C-peptide

78
Q

Insulin and Na - K

A

↑ Na retention and ↑ cellular uptake of K

79
Q

GLUT 1 receptor

A

RBCs, brain

80
Q

Cells which do not need insulin for glucose uptake

A

Brain, RBCs, intestine, cornea, kidney, liver

81
Q

Insulin

A

Dephosphorylates (↓cAMP → ↓PKA)

82
Q

Glucagon

A

Phosphorylates (↑cAMP → ↑PKA)

83
Q

Von Gierke’s disease (I)

A

Deficit of G-6-Pasa

84
Q

Von Gierke’s disease

A

Fasting hypoglycemia, ↑ blood lactate, ↑↑ glycogen in liver, hepatomegaly

85
Q

Pompe’s disease (II)

A

Cardiomegaly

86
Q

Pompe’s disease

A

Deficit of Lysosomal a-1,4-glucosidasa (acid maltasa)

87
Q

Cori’s disease (III)

A

Mild type I, blood lactate levels

88
Q

Cori’s disease

A

Deficit of a-1,6-glucosidase

89
Q

McArdle’s disease (V)

A

↑ glycogen in muscle

90
Q

McArdle’s disease

A

Deficit of skeletal muscle glycogen phosphorylase

91
Q

Fabry’s disease

A

a-galactosidase A - Ceramide trihexoside

92
Q

Gaucher’s disease

A

b-glucocerebrosidase - glucocerebroside

93
Q

Niemann-Pick disease

A

Sphingomyelinase - Sphingomyelin

94
Q

Tay-Sachs disease

A

Hexosaminidase A - GM2 ganglioside

95
Q

Krabbe’s disease

A

Galactocerebrosidasa - Galactocerebroside

96
Q

Metachromatic leukodystrophy

A

Arylsulfatase - Cerebroside sulfate

97
Q

Hurler’s sd

A

a-L-iduronidase - Heparan and dermatan sulfate

98
Q

Hunter’s sd

A

Irudonate sulfatase - Heparan and dermatan sulfate

99
Q

Carnitine deficiency

A

Inability to utilize LCFAs and toxic accumulation

100
Q

Acil-CoA DH deficiency

A

↑ dicarboxilic acids, ↓ glucose and ketones

101
Q

Chylomicron

A

A-IV, B-48, C-II, E

102
Q

VLDL

A

B-100, C-II, E

103
Q

IDL

A

B-100, E

104
Q

LDL

A

B-100

105
Q

Lead poisoning

A

Ferrochelatase and ALA dehydratase

106
Q

Acute intermittent porphyria

A

Porphobilinogen deaminasa

107
Q

Porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase

108
Q

Fetal Hb

A

2 alfa and 2 gamma

109
Q

Cyanide poisoning treatment

A

Nitrites and thiosulfate

110
Q

Methemoglobinemia treatment

A

Methylene blue

general knowledge medicine (9 decks)

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