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Biochemistry > Lipids > Flashcards

Lipids Flashcards

1
Q

Precursor of arachidonic acid

A

Linoleic acid

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2
Q

2 major sites of fatty acid synthesis

A

Liver and lactating mammary glands

Minor: adipose

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3
Q

Product of FA synthesis

A

Palmitate (16 carbons)

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4
Q

Transfer of acetyl CoA from the mitochondria to the cytoplasm is through the

A

Citrate shuttle

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5
Q

2 Activators of Acetyl CoA carboxylase

A

Insulin and citrate

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6
Q

How many acetyl CoA and Malonyl CoA and NADPH are needed for synthesis of palmitate?

A

1 Acetyl CoA
7 Malonyl CoA
14 NADPH

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7
Q

Sequence of steps in FA synthesis

A

Condensation
Reduction
Dehydration
Reduction

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8
Q

Nonessential FA may have double bonds that does not exceed the ____th carbon

A

9th

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9
Q

Active form of FA

A

Fatty acyl CoA

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10
Q

Main storage form of FA

A

TAG

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11
Q

2 Sources of glycerol-3-P for TAG

A

DHAP (liver and adipose)

Phosphorylation of free glycerol by glycerol kinase (in liver only)

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12
Q

Hydrolyzes TAGs to yielding free fatty acids and glycerol

A

Hormone sensitive lipase

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13
Q

4 organs/cells where beta oxidation does not occur

A

Neurons
RBCs
Testis
Renal medulla

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14
Q

3 Substrates of beta oxidation

A

Palmitate
NAD
FAD

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15
Q

Products of Beta oxidation

A

8 acetyl CoA
7 FADH2
7 NADH

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16
Q

Rate limiting step and enzyme in beta oxidation

A

Carnitine acyltransferase or carnitine palmitoyl transferase

(Fatty acyl CoA + carnitine –> fatty acyl carnitinr+ CoA

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17
Q

Shuttle for long chain FAs (>12 carbons) for beta oxidation

A

Carnitine shuttle

Short chain (2-4 C) and Medium chain (6-12) do not need shuttle

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18
Q

4 important steps in beta oxidation

A

Oxidation
Hydration
Oxidation
Thiolysis

Multiplied 7 times

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19
Q

Number of ATPs produced in beta oxidation of Palmitate

A

129 ATPs

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20
Q

Propionyl CoA is converted to

A

Succinyl CoA

Via 2 steps

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21
Q

Propionyl CoA to methylmalonyl CoA is catalyzed by ______ and requires ______

A

Propionyl CoA carboxylase; biotin

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22
Q

Methylmalonyl CoA to Succinyl CoA is catalyzed by ________ and requires_________

A

Methyl malonyl CoA mutase; cobalamin

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23
Q

Very long chain FAs are oxidized in the

A

Peroxisome

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24
Q

Caused by earing unripe fruit of the akee tree, which contains hypoglycin, a toxin that inactivates medium- and short chain acyl CoA dehydrogenase and leads to hypoglycemia

A

Jamaican Vomiting Sickness

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25
Q

Cerebrohepatorenal syndrome which occurs in individuals with a rare inherited absence of peroxisomes in all tissues

A

Zellweger’s syndrome

Jaundice, marked mental retardation, weakness, hypotonia, and craniofacial dysmorphism

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26
Q

Defect in peroxismal activation of VLCFA in the blood and tissues

A

X-linked adrenoleukodystrophy

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27
Q

Location of ketone bodies synthesis

A

Liver mitochondria

28
Q

Substrate for Ketone bodies synthesis

A

Acetyl CoA

29
Q

Rate-limiting step and enzyme in ketone bodies synthesis?

A

HMG CoA synthase

Acetoacetyl CoA + Acetyl CoA to HMG CoA

30
Q

Liver cannot use ketone bodies because they lack what enzyme?

A

Thiophorase (succinyl CoA acetoacetyl CoA transferase)

31
Q

Urine test for ketones

A

Nitroprusside test

Does not detect beta-hydroxybutyrate

32
Q

Deficiency of lipoprotein lipase

A

Type 1 or Familial lipoprotein lipase deficiency

High VLDL and chylomicrons
Low LDL and HDL

Xanthomas and pancreatitis
No increased risk of CAD

33
Q

Type 1 lipoproteinemia

A

Familial lipoprotein lipase deficiency

34
Q

Type II lipoproteinemia

A

Familial hypercholesterolemia

35
Q

Deficienct in LDL receptors

A

Type II or familial hypercholesterolemia

High LDL

36
Q

Type III lipoproteinemia

A

Familial dysbetalipoproteinemia

37
Q

Deficiency in apo-E

A

Type III or Familial dysbetalipoproteinemia

High remnants of VLDL and chylomicron

38
Q

Increased VLDL production

A

Type IV or Familial hypertriglyceridemia

39
Q

Type IV lipoproteinemia

A

Familial hypertriglyceridemia

40
Q

Deficiency in apoB48 and 100

A

Abetalipoproteinemia

Intestinal malabsorption

41
Q

Defeciency in apo A1

A

Tangier’s disease and Fisheye disease

No HDL

42
Q

High HDL

A

Familial hyperalphalipoproteinemia

43
Q

Predominant lipids of cell membrane

A

Phospholipids

44
Q

Phospholipid that plays a role in apoptosis

A

Phosphatidylserine

45
Q

Sphingosine + FA

A

Ceramide

46
Q

Ceramide + glucose or galactose

A

Cerebroside

47
Q

Ceramide + oligosaccharide

A

Globoside

48
Q

Ceramide + N-acetylneuraminic acid

A

Ganglioside

49
Q

Ceramid + sulfated galactose

A

Sulfatides

50
Q

Deficiency of hexosaminidase A

A

Tay-Sach’s disease

Cherry red macula, MR, and hypotonia

51
Q

Deficiency of alpha-galactosidase

A

Fabry’s disease

Rash and renal failure

52
Q

Deficiency of ceramidase

A

Farber’s disease

Skin rash, hoarseness, bone malformation

53
Q

Deficiency in arylsulfatase A

A

Metachromatic leukodystrophy

Physiologic disturbance in adults due to demyelination

54
Q

Deficiency in beta galactosidase

A

Krabbe’s disease

MR

55
Q

Deficiency in beta glucosidase

A

Gaucher’s disease

Hepatosplenomegaly and erosion of long bones

56
Q

Deficiency of sphingomyelinase

A

Niemann-Pick disease

Foam cells
Hepatosplenomegaly

57
Q

All lipid stirage disease are autosomal recessive except for

A

Fabry’s disease (x-linked recessive)

58
Q

Accumulation of GM2 ganglioside

A

Tay-Sachs disease

59
Q

Accumulation of sphingomyelin

A

Niemann-Pick disease

60
Q

Accumulation of ceramide

A

Farber’s disease

61
Q

What accumulates in Gaucher disease?

A

Glucosylceramide

62
Q

What accumulates in Krabbe’s disease?

A

Galactosylceramide

63
Q

Accumulation of globotriaosylceramide

A

Fabry’s disease

64
Q

Accumulation of 3-sulfogalactosylceramide

A

Metachromatic leukodystrophy

65
Q

Oligosaccharide in Type A blood

A

N-acetyl-galactosamine

66
Q

Oligosaccharide in Type B blood

A

Galactose

Biochemistry (6 decks)

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