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Spingomyelin
Niemann-Pick disease:
accumulation of spingomyelin in
the liver and spleen
Cephalin
DONT FORGET
Main Lipids
(human plasma)
Phospholipids
Cholesterol
Triglycerides
Amphipathic
Fatty acids
Phospholipids
Conjugated
CHOLESTEROL
Progestins, glucocorticoids,
mineralocoticoids, androgens, estrogens
Forms:
Lecithin/Phosphatidyl choline
Cholesterol Ester
Free Cholesterol
TRIGLYCERIDE
FATTY ACIDS
LCAT
Endothelial Lipase
LIPOPROTEIN
LIPOPROTEIN METABOLISM
Major function
Apolipoprotein
Electrophoretic mobility
LIPOLYTIC ENZYMES
LPL
Hepatic Lipase
Four Major
Lipoprotein Classes:
Chylomicrons
Produced by intestine
PRODUCTION: INTESTINES
VLDL
TURBID
PRODUCTION: LIVER
LDL
No Change in Appearance
Bad Cholesterol
PRODUCTION: LIVER
Density: 1.019-1.063
CHEMICAL COMPOSITION:
Cholesterol Transports cholesterol to
tissue
HDL
Good Cholesterol
Minor lipoproteins
LIPOPROTEIONS
ABNORMAL LIPOPROTEINS
Chylomicrons:
LpX
MILKY
apoB-48
ApoC
Different groups:
ApoC-I
ApoC-II
APOLIPOPROTEIN
ApoC-III
ApoE
ApoA
Major component:
ApoA-I
Minor apolipoprotein
ApoD
ApoA-IV
Activator of enzyme
Lecithin:Cholesterol
Acyltransferase (LCAT)
esterifies cholesterol in plasma
Cherubs Everywhere in
Heavens Above
ApoB
Chylomicrons = All
While
HDL = ApoA
Lucifer Below
LDL = ApoB
for he is
Several forms:
Very Bad
ApoA-II
VLDL = ApoB
apoB-100
Electrophoresis
Pattern: HDL, VLDL, LDL, Chylomicrons
Supporting Medium: agarose-gel
Disorders
Fredrickson Classification
TYPE 1: Hyperchylomicronemia
TYPE 2
TYPE 5
Familial Hypercholesterolemia
(Type 2a)
Tangier's Disease
LPL deficiency
Deficiency of ApoC-II
Hypobetalipoproteinemia
Abetalipoproteinemia (Bassen-Kornzweig
Syndrome)
LCAT deficiency
Tay-Sachs Disease
Characterized by hypocholesterolemia,
chronic diarrhea, deficiency of fat soluble
vitamins