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Biochem Flashcards

1
Q

Which Apolipoprotein mediates remnant uptake?

A

Apo E

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2
Q

Which Apolipoprotein activates LCAT for HDL maturation?

A

Apo-A1

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3
Q

Which Apolipoprotein is lipoprotein lipase cofactor? (Removes FFA from lipid particles)

A

Apo-C2

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4
Q

Which Apolipoprotein mediates chylomicron secretion from enterocytes?

A

Apo B-48

“Lipid from when you ate”

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5
Q

Which Apolipoprotein binds LDL/VLDL receptor?

A

Apo B-100

Allows the cholesterol to leave the liver

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6
Q

Which Apolipoprotein is deficient in A-beta lipoproteinemia?

A

Apo B-48

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7
Q

AR Lipoprotein lipase deficiency
Altered apolipoprotein C-II
Cause pancreatitis, hepatosplenomegaly, eruptive xanthomas

A

Familial dyslipidemia

Type 1 hypercholmicronemia

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8
Q

Familial dyslipidemia with increased blood levels of chylomicrons TG and cholesterol

A

Familial dyslipidemia

Type 1 hypercholmicronemia

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9
Q

Familial dyslipidemia with no risk for atherosclerosis

A

Familial dyslipidemia

Type 1 hypercholmicronemia

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10
Q

Familial dyslipidemia with increased blood levels of LDL and cholesterol

A

Type 2a familial hypercholesterolemia

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11
Q

Familial dyslipidemia with increased blood levels of VLDL and TG

A

Type 4 hypertriglyceridemia

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12
Q 
AD Absent or defective LDL receptors 
Accelerated atherosclerosis 
MI before age 20 
Tendon xanthomas 
Corneal arcus
A

Familial dyslipidemia

Type 2a familial hypercholesterolemia

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13
Q

AD Hepatic overproduction of VLDL
Hypertrigly >1000
Acute pancreatitis

A

Familial dyslipidemia

Type 4 hypertriglyceridemia

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14
Q

Acetoacetate and B-hydroxybutyrate are

A

Ketone bodies

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15
Q

Inherited defect in transport of long chain fatty acids into the mitochondria
Toxic accumulation causes weakness, hypotonia, hypoketotic hypoglycemia

A

Systemic primary carnitine deficiency

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16
Q

XR Deficient a-galactosidase A

Accumulated ceramide trihexoside

A

Fabry disease

Fairy wings make an X

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17
Q

AR Deficient glucocerebrosidase (B-glucosidase)

Accumulated glucocerebroside

A

Gaucher disease

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18
Q

Lipid laden macrophages (crumpled tissue paper)

A

Gaucher disease

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19
Q

AR Deficient sphingomyelinase

Accumulated spingomyelin

A

Niemann - Pick Disease

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20
Q

Foam cells

A

Niemann-Pick Disease

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21
Q

AR Deficient Hexosaminidase A

Accumulated GM2 ganglioside

A

Tay-Sachs Disease

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22
Q

AR Deficient Galactocerebrosidase

Accumulated Galctocerebroside & Psychosine

A

Krabbe Disease

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23
Q

AR Deficient Arylsulfatase A

Accumulated Cerebroside sulfate

A

Metachomatic leukodystrophy

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24
Q

AR Deficient a-L-iuronidase
Accumulated Heparan sulfate & Dermatan sulfate
Corneal clouding

A

Hurler Syndrome

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25
XR Deficient Iduronate sulfatase Accumulated Heparin sulfate & Dermatan suflate Milder with aggressive behavior and no corneal clouding
Hunter Syndrome | X marks the spot for hunters
26
Which enzyme in the Krebs Cycle produces GTP?
Succinyl CoA Synthetase
27
Fructose 2,6- Bisphosphate effects on Glycolysis and Gluconeogenesis
Promotes PFK-1 in glycolysis | Inhibits Fructose 1,6 bisphosphatase in Gluconeogensis
28
``` Deficient Glucose 6-phophate Severe fasting hypoglycemia Increased glycogen in liver Increased blood lactate Increased triglycerides Increased uric acid Heaptosplenomegaly ```
Von Gierke | Glycogen Storage Disease
29
Tx frequent oral glucose/cornstarch
Von Gierke type 1 | Glycogen Storage Disease
30
Deficient lysosomal a-1,4-glucosidase (acid maltase)
Pompe type 2 | Glycogen Storage Disease
31
Cardiomegaly Exercise intolerance Early death with infantile form
Pompe type 2 | Glycogen Storage Disease
32
Deficient debranching enzyme (a-1,6-glucosidase) Mild hypoglycemia Normal blood lactate levels
Cori disease type 3 Glycogen Storage Disease (hepatomegaly, ketoacidosis and hypotonia)
33
``` Deficient skeletal muscle glycogen phosphorylase (myophosphorylase) Painful muscle cramps and myoglobinuria Arrythmia Normal blood glucose levels Tx with Vit B6 ```
McArdle type 5 | Glycogen Storage Disease
34
Cytosolic accumulation of glycogen with short outer chains (limit dextrans)
Cori disease type 3 | Glycogen Storage Disease
35
Enzymes requiring TLC For Nancy
Pyruvate Dehydrgonase a-Ketoglutarate dehydrogenase a-Ketoacid dehydrogenase
36
Enzymes inhibited in lead toxicity
d-Aminolevulinate dehydratase Ferrochelatase Enzymes in heme synthesis
37
Homocytinuria Tx
Decreased methionine | Increased Vit B6 and Pyridoxine
38
Required for synthesis of ofrhodopsin
Vit A (Retinol)
39
Required for synthesis of NAD/NADP
Vit B3 (Niacin)
40
Required for synthesis of Niacin (Vit B3)
Tryptophan
41
Required for synthesis of FAD FAMN
Riboflavin (Vit B2)
42
Required for synthesis of TPP
Thiamine (VitB1)
43
Hydroxylation of proline and lysine in synthesis of collage
Vit C (Ascorbic Acid)
44
Deficiency seen in vegans
Vit B12 Cobalamin
45
Cofactor in methylmalonyl CoA to succinyl CoA | Cofactor conversion of homocysteine to methionine
Vit B12 Cobalamin
46
Required for synthesis of thymidylate Required for conversion of homocysteine to methionine Labs show increased homocysteine
Folic Acid Vit B9
47
Cofactor for pyruvate carboxylase and proprionyl CoA carboxylase and acetyl CoA carboxylase
Biotin B7 deficiency
48
Deficiency of HGPRT Hyperuricemia Part of purine salvage pathway
Lesch-Nyhan syndrome
49
Mutation of sodium dependent transport channel of neutral amino acids Relative deficiency in tryptophan
Hartnup Disease
50
Deficiency of URP decarboxylase
Porphyria Cutanea Tarda
51
Required for synthesis of transamination reaction cofactor for cystathionine synthase and heme
Vit B6 pyridoxine
52
Required for synthesis of cysathionine, heme, niacin, histamine, serotonin, Epi, NE, Dopa GABA
Vit B6 pyridoxine
53
Cofactor for metalloenzymes
Zinc deficiency
54
Mutation in fibrillin gene
Marfans
55
Increased CTG repeats in myotonic protein kinase
Myotonic dystrophy
56
Frameshift mutation | Absence of dystrophin synthesis
Duchenne Muscular Dystrophy
57
Increased serum creatine kinase
Duchenne Muscular Dystrophy
58
Pseudo hypertrophy of calves
Duchenne Muscular Dystrophy
59
Gowers manuevere
Duchenne Muscular Dystrophy
60
Reduced synthesis of dystrophin
Becker Muscular Dystrophy
61
Pyruvate dehydrogenase complex deficiency Tx
Lysine and leucine
62
Regenerates NADPH
Glucose 6 phosphate dehydrogenase
63
Heinz bodies and bite cells
Glucose 6 phosphate dehydrogenase
64
Maintains glutathione in its reduced form
Glucose 6 phosphate dehydrogenase
65
Deficiency in fructokinase
Essential Fructosuria | "kind disease"
66
Deficiency in fructose- 1,6-bisphosphate aldolase B
Fructose intolerance Causes impaired glycolysis, glucogenolysis and gluconeogeneisis "A bad disease"
67
Deficiency of galactokinase | Galactitol builds up in eye
Galactokinase deficinecy | "kind disease"
68
Deficiency of galactose-1-phosphate uridyl transferase
Classic Galactosemia | "GUT" enzyme
69
Lactase deficiency can be induced by
Gastroenteritis
70
Mutation in UMP synthase of pyrimidine synthesis | Normal ammonia
Orotic aciduria
71
Mutation in ornithine transcarbamylase Increased aortic acid Increased ammonia
Ornithine transcarbamylase deficiency
72
Mutation in phenylalanine hydroxylase | Decreased tetrahydrobiopterin
Phenylketonuria
73
Mutation in enzyme converting phenylalanine into tyrosine
Phenylketonuria
74
Tx PKU
Increased dietary tyrosine | Decreased dietary phenylalanine
75
Deficiency in homogentisic acid oxidase
Alkaptonuria
76
Defect in cystathionine synthase Defect in homocysteine methyltransferase Decreased affinity of cystathionine synthase for Vit B6
Homocystinuria
77
Tx Homocystinuria
Decreased methionine | High dose Vit B6
78
Subluxation of lens down and in Thromboembolism Coronary artery disease
Homocystinuria
79
a-ketoacid dehydrogenase deficiency
Maple Syrup Urine Disease
80
Defective isoleucine, leucine, valine metabolism
Maple Syrup Urine Disease

DIT/Boards (22 decks)

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