biochemie USMLE Flashcards
1
Q
Carried out in cytoplasm
A
Glycolisis, HMP shunt, fatty acid and steroid synthesis
2
Q
Carried out in mitochondria
A
b-oxidation, acetyl-Co A production, Krebs cycle, oxidative phosphorylation
3
Q
De novo pyrimidine synthesis
A
ATCase
4
Q
Carried out in cytoplasm and mitochondria
A
Heme synthesis, Urea cycle, Gluconeogenesis
5
Q
De novo purine synthesis
A
Glutamine-PRPP amidotransferasa
6
Q
Glycolysis
A
PFK-1
7
Q
Gluconeogenesis
A
F 1,6 BP
8
Q
TCA cycle
A
Isocitrate DH
9
Q
Glycogen synthesis
A
Glycogen synthasa
10
Q
Glycogenolysis
A
Glycogen phosphorylase
11
Q
HMP shunt
A
G6PDH
12
Q
Fatty acid S.
A
ACC
13
Q
Fatty acid O.
A
Carnitine acyltranferase I
14
Q
Ketogenesis
A
HMG-Co A synthase
15
Q
Cholesterol synthesis
A
HMG-Co A reductase
16
Q
Heme S.
A
ALA synthase
17
Q
Urea cycle
A
Carbamoyl phosphate synthase
18
Q
Aerobic metabolism of glucose
A
30-32 ATP
19
Q
Anaerobic glycolysis
A
2 ATP
20
Q
CO2 carrier
A
Biotin
21
Q
l-carbon units carrier
A
THF
22
Q
Aldehydes
A
TPP
23
Q
Uses of NADPH
A
Anabolic processes, respiratory burst, P-450
24
Q
Hexokinase
A
Ubiquitous, high affinity, low capacity, uninduced by insulin, feedback by G-6-P
25
F 2,6 BP
Most potent activator of PFK
26
Glycolitic enzime deficiency
Hemolytic anemia (↓ Na K ATPasa activity)
27
Cofactors of Pyruvate and a-ketoglutarate DH complex
B1, B2, B3, B5 and lipoic acid (inhibited by arsenic)
28
Ketogenic aa
Lysine Leucine
29
Cory cycle sites
Liver → Muscle/RBCs
30
Cory cycle
Hepatic gluconeogenesis of lactate
31
ATP asa inhibitors
Oligomycin
32
Uncoupling agents
2,4 DNP, aspirin, termogenin
33
e- transport inhibitors
Rotenone, CN-, antimycin A, CO
34
Gluconeogenesis, irreversible enzimes
Pyruvate carboxylase, PEP carboxykinase, F 1,6 BP, G 6 P
35
HMP shunt product
NADPH
36
Fructose intolerance-symptoms
Hypoglicemia, jaundice, cirrhosis, vomiting
37
G 6 P DH deficiency-symptoms
Hemolytic anemia, heinz bodies, X-linked recessive
38
Aldolase B deficiency
Fructose intolerance
39
Essential fructosuria-
Defect in fructokinase
40
Galactosemia-symptoms
Cataracts, hepatosplenomegaly, mental retardation
41
Galactosemia
Abscence of Galactose-1-phosphate uridyltranferase
42
Lactase deficiency-symptoms
Bloating, cramps, osmotic diarrhea
43
Glucogenic/Ketogenic aa
Phe, Thr, Trp, ILe
44
Glucoegnic aa
Va, Met, Arg, His
45
Ammonia intoxication-symptoms
Tremor, slurred speech, somnolence, vomiting, cerebral edema, blurred vision
46
Ammonia intoxication-Treatment
Benzoate or phenylbutirate
47
Urea cycle, aa related
Aspartate, arginine
48
Phenylalanine derivatives
Tyrosine (thyroxine), DOPA (melanin), dopamine, NE, epi
49
Tryptophan derivatives
Niacin (NAD/NADP), serotonin-melatonin
50
Histidine derivatives
Histamine
51
Glycine derivatives
Porphyrin-Heme
52
Arginine derivatives
Creatine, urea, NO
53
Glutamate derivatives
GABA, glutathione
54
Phenylketonuria, symptoms
Autosomal recessive. Mental and growth retardation, fair skin, eczema, musty body odor
55
Phenylketonuria, defects
↓ phenylalanine hydroxilase or ↓tetrahidrobiopterin cofactor
56
Tyrosine food sources
Meat, fish, milk, wheat, oats, leafy vegetables,bananas, nuts
57
Alkaptonuria, defect
Homogentisic acid oxidase deficiency
58
Alkaptonuria, symptoms
Arthralgias, dark connective tisue, urine to turn black on standing, bening disease
59
Albinism, defects
Deficiency of tyrosinase or its transporters
60
Homocystinuria, signs
Autosomal recessive. Mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation, atherosclerosis
61
Homocystinuria, deficiencies
Cystathion synthase or homocysteine methyltranferase. ↓ affinity of cystathion synthase for pyridoxal phosphate)
62
Cystinuria, defect
Transporter defect of Cys Orn Lys Arg
63
Staghorn calculi
Cystinuria (AR)
64
Maple syrup urine disease (AR)
Blocked degradation of branched aa due to ↓ a-ketoacid DH
65
Branched aa
Ile Leu Val
66
Maple syrup urine disease
CNS defects, mental retardation, urine smells like maple syrup
67
Purine salvatage deficiencies
ADA deficiency, Lesh-Nyhan sd.
68
ADA deficiency
Can cause SCID
69
Lesh-Nyhan sd.
Absence of HGPRT (converts hipoxanthine→IMP and guanine→GMP)
70
Lesh-Nyhan sd.
Retardation, self mutilation, aggression, hyperuricemia, gout, choreoathetosis
71
100-meter sprint
Stored ATP, creatine phosphate, anaerobic glycolysis
72
100-meter run
Stored ATP, creatine phosphate, anaerobic glycolysis, oxidative phosphorylation
73
Marathon
Glycogen, FFA oxidation. Glucose for final sprinting
74
Starvation after day 3
Hepatic formation of ketone bodies
75
GLUT 2 receptor
b cells, liver, kidney
76
GLUT 4 receptor (insuline responsive)
muscle and fat
77
Proinsulin
insulin + C-peptide
78
Insulin and Na - K
↑ Na retention and ↑ cellular uptake of K
79
GLUT 1 receptor
RBCs, brain
80
Cells which do not need insulin for glucose uptake
Brain, RBCs, intestine, cornea, kidney, liver
81
Insulin
Dephosphorylates (↓cAMP → ↓PKA)
82
Glucagon
Phosphorylates (↑cAMP → ↑PKA)
83
Von Gierke's disease (I)
Deficit of G-6-Pasa
84
Von Gierke's disease
Fasting hypoglycemia, ↑ blood lactate, ↑↑ glycogen in liver, hepatomegaly
85
Pompe's disease (II)
Cardiomegaly
86
Pompe's disease
Deficit of Lysosomal a-1,4-glucosidasa (acid maltasa)
87
Cori's disease (III)
Mild type I, blood lactate levels
88
Cori's disease
Deficit of a-1,6-glucosidase
89
McArdle's disease (V)
↑ glycogen in muscle
90
McArdle's disease
Deficit of skeletal muscle glycogen phosphorylase
91
Fabry's disease
a-galactosidase A - Ceramide trihexoside
92
Gaucher's disease
b-glucocerebrosidase - glucocerebroside
93
Niemann-Pick disease
Sphingomyelinase - Sphingomyelin
94
Tay-Sachs disease
Hexosaminidase A - GM2 ganglioside
95
Krabbe's disease
Galactocerebrosidasa - Galactocerebroside
96
Metachromatic leukodystrophy
Arylsulfatase - Cerebroside sulfate
97
Hurler's sd
a-L-iduronidase - Heparan and dermatan sulfate
98
Hunter's sd
Irudonate sulfatase - Heparan and dermatan sulfate
99
Carnitine deficiency
Inability to utilize LCFAs and toxic accumulation
100
Acil-CoA DH deficiency
↑ dicarboxilic acids, ↓ glucose and ketones
101
Chylomicron
A-IV, B-48, C-II, E
102
VLDL
B-100, C-II, E
103
IDL
B-100, E
104
LDL
B-100
105
Lead poisoning
Ferrochelatase and ALA dehydratase
106
Acute intermittent porphyria
Porphobilinogen deaminasa
107
Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
108
Fetal Hb
2 alfa and 2 gamma
109
Cyanide poisoning treatment
Nitrites and thiosulfate
110
Methemoglobinemia treatment
Methylene blue
