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Biochemistry > Lipids > Flashcards

Lipids Flashcards

1
Q

Generally compartmentalized to protect themselves from watery environment of cells
Transported in blood by lipoproteins

A

Lipids

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2
Q

Long chains of carboxylic acids

A

Fatty acids

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3
Q

FA: Predominant in coconut oil

A

Lauric Acid

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4
Q

End product of mammalian FA synthesis

A

Palmitic Acid

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5
Q

Predominant FA in olive oil

A

Oleic acid

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6
Q

Essential Fatty Acids

A

Linoleic acid

Linolenic acid

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7
Q

Precursor of prostaglandins, derived from linoleic acid

A

Arachidonic acid

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8
Q

Synthesis of fatty acids, specifically, palmitate

A

Lipogenesis

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9
Q

Where does Lipogenesis occur?

A

Liver

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10
Q

Immediate substrate of Lipogenesis

A

Acetyl CoA

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11
Q

Product of Lipogenesis

A

Palmitoyl CoA

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12
Q

Rate limiting step of Lipogenesis

A

Acetyl CoA carboxylase

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13
Q

Transport of Acetyl CoA to the Cytosol via

A

Citrate Shuttle

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14
Q

Identify the enzyme: Acetyl CoA is converted to Malonyl CoA

A

Acetyl CoA carboxylase

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15
Q

The rate limiting step of lipogenesis is activated by

A

Citrate

Insulin

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16
Q

The rate limiting step of lipogenesis is inhibited by

A

Glucagon

Epinephrine

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17
Q

Fatty acids are stored as

A

Triacylglycerol

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18
Q

Sources of Glycerol-3-Phosphate

A

DHAP from glycolysis

Phosphorylation of free glycerol

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19
Q

Identify the enzyme: DHAP from glycolysis

A

Glycerol 3 phosphate dehydrogenase (liver and muscle)

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20
Q

Identify the enzyme: Phosphorylation of free glycerol

A

Glycerol kinase (liver only)

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21
Q

Identify the enzyme: Triacylglycerol -> Glycerol + 3 Fatty Acids

A

Hormone sensitive lipase

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22
Q

Removal of Acetyl CoA fragments from the ends of fatty acids, also yielding NADH and FADH2 in the process

A

Beta-Oxidation

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23
Q

Substrate of Beta-Oxidation

A

Palmitate

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24
Q

Products of Beta-Oxidation

A

8 Acetyl CoA
7 NADH
7 FADH2

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25
Q

Rate limiting step of Beta-Oxidation

A

Carnitine-Palmitoyl transferase

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26
Q

Sequence of Elongation to Palmitoyl CoA

A

Condensation
Reduction
Dehydration
Reduction

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27
Q

Sequence of Degradation of Fatty Acyl CoA

A

Oxidation
Hydration
Oxidation
Thiolysis

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28
Q

Propionyl CoA carboxylase requires

A

Biotin

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29
Q

Methylmalonyl CoA mutase requires

A

Vitamin B12

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30
Q

____________ oxidize very long chains of fatty acids

A

Peroxisomes

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31
Q

Steatorrhea

A

Lipid Malabsorption

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32
Q

Results in deficiency in fat soluble vitamins and essential fatty acids

A

Lipid Malabsorption

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33
Q

Ichthyosis (Scaly dermatitis)
Hair loss
Poor wound healing

A

Deficiency of Essential Fatty Acids

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34
Q

Inadequate synthesis or renal loss of carnitine

A

Carnitine deficiency

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35
Q

Clinical presentation of Carnitine deficiency

A

Hypoglycemia - impaired fatty acid oxidation

Lipid accumulation with muscular weakness

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36
Q

Treatment of Carnitine deficiency

A

Oral supplementation with carnitine

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37
Q

Due to reduced fatty acid oxidation, leading to severe hypoglycemia, coma, and even death
Affects the LIVER

A

Carnitine-Palmitoyl Transferase I deficiency

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38
Q

Cardiomyopathy
Muscle weakness with myoglobinemia after prolonged exercise
Affects CARDIAC and SKELETAL MUSCLE

A

Carnitine-Palmitoyl Transferase II deficiency

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39
Q

Most common inborn error of fatty acid oxidation

A

Medium-Chain Fatty Acyl CoA Dehydrogenase (MCAD)

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40
Q

Severe hypoglycemia
Accumulation of fatty acids in the urine
Can manifest Sudden Infant Death Syndrome

A

Medium-Chain Fatty Acyl CoA Dehydrogenase Deficiency (MCAD)

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41
Q

Treatment of MCAD

A

IV glucose during acute episodes

Avoidance of fasting

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42
Q

Caused by eating the unripe fruit of the akee tree

A

Jamaican Vomiting Sickness

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43
Q

Deficiency of Phytanoyl-CoA hydroxylase

Accumulation of Phytanic Acid

A

Refsum Disease

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44
Q

Clinical presentation of Refsum Disease

A

Peripheral neuropathy and ataxia
Retinitis pigmentosa
Abnormalities of the skin and bones

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45
Q

Treatment of Refsum disease

A

Diet low in phytanic acid

Avoid dairy products, ruminant fat and meat

46
Q

Also known as Cerebrohepatorenal Syndrome

A

Zellweger syndrome

47
Q

Inherited absences of peroxisomes in all tissues -> accumulation of very long chain, saturated, unbranched fatty acids (liver and CNS)

A

Zellweger Syndrome

48
Q

Inability to transport VLCFAa across the peroximal membrane leads to accumulation in the brain, adrenals, and testes

A

Adrenoleukodystrophy

49
Q

Clinical Presentation of Adrenoleukodystrophy

A

Neurodegeneration
Adrenocortical insufficiency
Hypogonadism

50
Q

Treatment for Adrenoleukodystrophy

A

Adrenal hormone replacement theraphy

Hematopoietic stem cell transplantation

51
Q

Synthesis of ketone bodies, which serve as alternative fuel for peripheral tissues

A

Ketogenesis

52
Q

Where does ketogenesis occur?

A

Liver mitochondria

53
Q

Substrate of Ketogenesis

A

Acetyl CoA

54
Q

Products of Ketogenesis

A

Acetoacetate
B-hydroxybutyrate
Acetone

55
Q

Rate limiting step of Ketogenesis

A

HMG CoA Synthase

56
Q

The liver is not able to use ketone bodies as fuel because it lacks the enzyme

A

Succinyl CoA-acetoacetate-CoA transferase

57
Q

Higher than normal levels of ketone bodies in the blood or urine

A

Ketosis

58
Q

In prolonged starvation and diabetic ketoacidosis, ________ is depleted for Gluconeogenesis

A

Oxaloacetate

59
Q

In alcoholism, _____ shunts oxaloacetate to malate

A

Excess NADH

60
Q

Measures both acetone and acetoacetate

A

Sodium nitroprusside reaction

61
Q

An important steroid in animal tissues

A

Cholesterol

62
Q

Cholesterol stored as

A

Cholesteryl esters

63
Q

Primary Bile Acids

A

Cholic acid

Chenodeoxycholic acid

64
Q

Secondary Bile Acids

A

Deoxycholic acid

Lithocholic acid

65
Q

Bile salts conjugate to

A

Taurine

Glycine

66
Q

Identify the enzyme: Cholesterol -> 7a-hydroxycholesterol

A

7a-hydroxylase

67
Q

Identify the enzyme: Cholesterol -> Pregnenolone

A

Desmolase

68
Q

Substrate of Cholesterol Synthesis

A

Acetyl CoA

69
Q

Rate limiting enzyme of Cholesterol Synthesis

A

HMG-CoA reductase

70
Q

HMG-CoA reductase requirement

A

2 NADPH

71
Q

Treatment for hypercholesterolemia

A

Statins

72
Q

Statins?

A

Reduce risk for cardiovascular disease

73
Q

Statins are

A

Competitive inhibitors of HMG-CoA reductase

74
Q

Precipitation of cholesterol in the gallbladder

A

Cholelithiasis

75
Q

Mor cholesterol enters the bile that can be solubilized the bile salts and phosphatidyl choline present

A

Cholelithiasis

76
Q

2 types of CAH

A

21-a-Hydroxylase deficiency

11-B1-Hydroxylase deficiency

77
Q

Most common form of CAH

A

21-a-Hydroxylase deficiency

78
Q

21-a Hydroxylase deficiency

A

Mineralocorticoids

Glucocorticoids are absent

79
Q

Decrease in serum cortisol, aldosterone, and corticosterone

A

11-B1- Hydroxylase deficiency

80
Q

Increased production of deoxycorticosterone causes

A

Fluid retention (Low renin hypertension)

81
Q

Increased production of deoxycorticosterone

A

11-B1- Hydroxylase deficiency

82
Q

Spherical macromolecular complexes of lipids and proteins called

A

Apolipoproteins

83
Q

The core of Plasma lipoproteins consist of

A

Neutral lipids

84
Q

The shell of plasma lipoproteins consists of

A

Amphiphatic apolipoproteins
Phospholipids
Free fatty acids

85
Q

Largest diameter
Lowest density
Highest TAG content

A

Chylomicron

86
Q

Highest Cholesterol content

A

LDL

87
Q

Highest protein content

A

HDL

88
Q

Foundly mainly in HDL

A

Apo A-1

89
Q

Cofactor of lecithin

A

Cholesterol acyltransferase

90
Q

Found mainly in VLDL, IDL, LDL

A

Apo B-100

91
Q

Main apoprotein

Mediates secretion of VLDL

A

Apo B-100

92
Q

Found mainly in chylomicrons

A

Apo B-48

93
Q

Main apoprotein

Mediates secretion of chylomicrons

A

Apo B-48

94
Q

Found mainly n HDL, VLDL, Chylomicrons

A

Apo C-II

95
Q

Cofactor of lipoprotein lipase

A

Apo C-II

96
Q

Found mainly in Chylomicrons, ILDL

A

Apo E

97
Q

Mediates uptake of chylomicrons remnants and IDLs

A

Apo E

98
Q

Imbalance between hepatic triacylglycerol synthesis and VLDL secretion

A

Fatty Liver

99
Q

Leads to increased synthesis of triacylglycerols

A

Chronic alcohol consumption

100
Q

Fatty liver can progress to

A

Alcoholic hepatitis

Alcoholic cirrhosis

101
Q

Deposition of cholesterol and cholesteryl esters in the artery walls especially from oxidized LDL

A

Atherosclerosis

102
Q

_________ cause endothelial damage which predisposes to atherosclerosis

A

Oxidized LDLs

103
Q

Mor severe in DM, lipid nephrosis, hypothyroidism

A

Atherosclerosis

104
Q

Deficiency in Lipoprotein lipase or Apo C-II

A

Type 1 Familial Lipoprotein Lipase Deficiency

105
Q

Defective LDL receptor

A

Type IIa Familial Hypercholesterolemia

106
Q

Abnormal Apo E

A

Type III Familial Dysbetalipoproteinemia

107
Q

Overproduction of VLDL

A

Type IV Familial Hypertriacylglycerolemia

108
Q

High HDL

A

Familial Hyperalpha-lipoproteinemia

109
Q

No chylomicrons or VLDL

A

Abetalipoproteinemia

110
Q

Low or near absence of HDL

A

Familial alpha-lipoprotein deficiency

Biochemistry (10 decks)

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