Lipids Flashcards
1
Q
Generally compartmentalized to protect themselves from watery environment of cells
Transported in blood by lipoproteins
A
Lipids
2
Q
Long chains of carboxylic acids
A
Fatty acids
3
Q
FA: Predominant in coconut oil
A
Lauric Acid
4
Q
End product of mammalian FA synthesis
A
Palmitic Acid
5
Q
Predominant FA in olive oil
A
Oleic acid
6
Q
Essential Fatty Acids
A
Linoleic acid
Linolenic acid
7
Q
Precursor of prostaglandins, derived from linoleic acid
A
Arachidonic acid
8
Q
Synthesis of fatty acids, specifically, palmitate
A
Lipogenesis
9
Q
Where does Lipogenesis occur?
A
Liver
10
Q
Immediate substrate of Lipogenesis
A
Acetyl CoA
11
Q
Product of Lipogenesis
A
Palmitoyl CoA
12
Q
Rate limiting step of Lipogenesis
A
Acetyl CoA carboxylase
13
Q
Transport of Acetyl CoA to the Cytosol via
A
Citrate Shuttle
14
Q
Identify the enzyme: Acetyl CoA is converted to Malonyl CoA
A
Acetyl CoA carboxylase
15
Q
The rate limiting step of lipogenesis is activated by
A
Citrate
Insulin
16
Q
The rate limiting step of lipogenesis is inhibited by
A
Glucagon
Epinephrine
17
Q
Fatty acids are stored as
A
Triacylglycerol
18
Q
Sources of Glycerol-3-Phosphate
A
DHAP from glycolysis
Phosphorylation of free glycerol
19
Q
Identify the enzyme: DHAP from glycolysis
A
Glycerol 3 phosphate dehydrogenase (liver and muscle)
20
Q
Identify the enzyme: Phosphorylation of free glycerol
A
Glycerol kinase (liver only)
21
Q
Identify the enzyme: Triacylglycerol -> Glycerol + 3 Fatty Acids
A
Hormone sensitive lipase
22
Q
Removal of Acetyl CoA fragments from the ends of fatty acids, also yielding NADH and FADH2 in the process
A
Beta-Oxidation
23
Q
Substrate of Beta-Oxidation
A
Palmitate
24
Q
Products of Beta-Oxidation
A
8 Acetyl CoA
7 NADH
7 FADH2
25
Rate limiting step of Beta-Oxidation
Carnitine-Palmitoyl transferase
26
Sequence of Elongation to Palmitoyl CoA
Condensation
Reduction
Dehydration
Reduction
27
Sequence of Degradation of Fatty Acyl CoA
Oxidation
Hydration
Oxidation
Thiolysis
28
Propionyl CoA carboxylase requires
Biotin
29
Methylmalonyl CoA mutase requires
Vitamin B12
30
____________ oxidize very long chains of fatty acids
Peroxisomes
31
Steatorrhea
Lipid Malabsorption
32
Results in deficiency in fat soluble vitamins and essential fatty acids
Lipid Malabsorption
33
Ichthyosis (Scaly dermatitis)
Hair loss
Poor wound healing
Deficiency of Essential Fatty Acids
34
Inadequate synthesis or renal loss of carnitine
Carnitine deficiency
35
Clinical presentation of Carnitine deficiency
Hypoglycemia - impaired fatty acid oxidation
| Lipid accumulation with muscular weakness
36
Treatment of Carnitine deficiency
Oral supplementation with carnitine
37
Due to reduced fatty acid oxidation, leading to severe hypoglycemia, coma, and even death
Affects the LIVER
Carnitine-Palmitoyl Transferase I deficiency
38
Cardiomyopathy
Muscle weakness with myoglobinemia after prolonged exercise
Affects CARDIAC and SKELETAL MUSCLE
Carnitine-Palmitoyl Transferase II deficiency
39
Most common inborn error of fatty acid oxidation
Medium-Chain Fatty Acyl CoA Dehydrogenase (MCAD)
40
Severe hypoglycemia
Accumulation of fatty acids in the urine
Can manifest Sudden Infant Death Syndrome
Medium-Chain Fatty Acyl CoA Dehydrogenase Deficiency (MCAD)
41
Treatment of MCAD
IV glucose during acute episodes
| Avoidance of fasting
42
Caused by eating the unripe fruit of the akee tree
Jamaican Vomiting Sickness
43
Deficiency of Phytanoyl-CoA hydroxylase
| Accumulation of Phytanic Acid
Refsum Disease
44
Clinical presentation of Refsum Disease
Peripheral neuropathy and ataxia
Retinitis pigmentosa
Abnormalities of the skin and bones
45
Treatment of Refsum disease
Diet low in phytanic acid
| Avoid dairy products, ruminant fat and meat
46
Also known as Cerebrohepatorenal Syndrome
Zellweger syndrome
47
Inherited absences of peroxisomes in all tissues -> accumulation of very long chain, saturated, unbranched fatty acids (liver and CNS)
Zellweger Syndrome
48
Inability to transport VLCFAa across the peroximal membrane leads to accumulation in the brain, adrenals, and testes
Adrenoleukodystrophy
49
Clinical Presentation of Adrenoleukodystrophy
Neurodegeneration
Adrenocortical insufficiency
Hypogonadism
50
Treatment for Adrenoleukodystrophy
Adrenal hormone replacement theraphy
| Hematopoietic stem cell transplantation
51
Synthesis of ketone bodies, which serve as alternative fuel for peripheral tissues
Ketogenesis
52
Where does ketogenesis occur?
Liver mitochondria
53
Substrate of Ketogenesis
Acetyl CoA
54
Products of Ketogenesis
Acetoacetate
B-hydroxybutyrate
Acetone
55
Rate limiting step of Ketogenesis
HMG CoA Synthase
56
The liver is not able to use ketone bodies as fuel because it lacks the enzyme
Succinyl CoA-acetoacetate-CoA transferase
57
Higher than normal levels of ketone bodies in the blood or urine
Ketosis
58
In prolonged starvation and diabetic ketoacidosis, ________ is depleted for Gluconeogenesis
Oxaloacetate
59
In alcoholism, _____ shunts oxaloacetate to malate
Excess NADH
60
Measures both acetone and acetoacetate
Sodium nitroprusside reaction
61
An important steroid in animal tissues
Cholesterol
62
Cholesterol stored as
Cholesteryl esters
63
Primary Bile Acids
Cholic acid
| Chenodeoxycholic acid
64
Secondary Bile Acids
Deoxycholic acid
| Lithocholic acid
65
Bile salts conjugate to
Taurine
| Glycine
66
Identify the enzyme: Cholesterol -> 7a-hydroxycholesterol
7a-hydroxylase
67
Identify the enzyme: Cholesterol -> Pregnenolone
Desmolase
68
Substrate of Cholesterol Synthesis
Acetyl CoA
69
Rate limiting enzyme of Cholesterol Synthesis
HMG-CoA reductase
70
HMG-CoA reductase requirement
2 NADPH
71
Treatment for hypercholesterolemia
Statins
72
Statins?
Reduce risk for cardiovascular disease
73
Statins are
Competitive inhibitors of HMG-CoA reductase
74
Precipitation of cholesterol in the gallbladder
Cholelithiasis
75
Mor cholesterol enters the bile that can be solubilized the bile salts and phosphatidyl choline present
Cholelithiasis
76
2 types of CAH
21-a-Hydroxylase deficiency
| 11-B1-Hydroxylase deficiency
77
Most common form of CAH
21-a-Hydroxylase deficiency
78
21-a Hydroxylase deficiency
Mineralocorticoids
| Glucocorticoids are absent
79
Decrease in serum cortisol, aldosterone, and corticosterone
11-B1- Hydroxylase deficiency
80
Increased production of deoxycorticosterone causes
Fluid retention (Low renin hypertension)
81
Increased production of deoxycorticosterone
11-B1- Hydroxylase deficiency
82
Spherical macromolecular complexes of lipids and proteins called
Apolipoproteins
83
The core of Plasma lipoproteins consist of
Neutral lipids
84
The shell of plasma lipoproteins consists of
Amphiphatic apolipoproteins
Phospholipids
Free fatty acids
85
Largest diameter
Lowest density
Highest TAG content
Chylomicron
86
Highest Cholesterol content
LDL
87
Highest protein content
HDL
88
Foundly mainly in HDL
Apo A-1
89
Cofactor of lecithin
Cholesterol acyltransferase
90
Found mainly in VLDL, IDL, LDL
Apo B-100
91
Main apoprotein
| Mediates secretion of VLDL
Apo B-100
92
Found mainly in chylomicrons
Apo B-48
93
Main apoprotein
| Mediates secretion of chylomicrons
Apo B-48
94
Found mainly n HDL, VLDL, Chylomicrons
Apo C-II
95
Cofactor of lipoprotein lipase
Apo C-II
96
Found mainly in Chylomicrons, ILDL
Apo E
97
Mediates uptake of chylomicrons remnants and IDLs
Apo E
98
Imbalance between hepatic triacylglycerol synthesis and VLDL secretion
Fatty Liver
99
Leads to increased synthesis of triacylglycerols
Chronic alcohol consumption
100
Fatty liver can progress to
Alcoholic hepatitis
| Alcoholic cirrhosis
101
Deposition of cholesterol and cholesteryl esters in the artery walls especially from oxidized LDL
Atherosclerosis
102
_________ cause endothelial damage which predisposes to atherosclerosis
Oxidized LDLs
103
Mor severe in DM, lipid nephrosis, hypothyroidism
Atherosclerosis
104
Deficiency in Lipoprotein lipase or Apo C-II
Type 1 Familial Lipoprotein Lipase Deficiency
105
Defective LDL receptor
Type IIa Familial Hypercholesterolemia
106
Abnormal Apo E
Type III Familial Dysbetalipoproteinemia
107
Overproduction of VLDL
Type IV Familial Hypertriacylglycerolemia
108
High HDL
Familial Hyperalpha-lipoproteinemia
109
No chylomicrons or VLDL
Abetalipoproteinemia
110
Low or near absence of HDL
Familial alpha-lipoprotein deficiency
