Lipoprotein Metabolism + Lipoprotein Disorders

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Author:
mstengs
ID:
188795
Filename:
Lipoprotein Metabolism + Lipoprotein Disorders
Updated:
2012-12-11 18:12:58
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Lipoprotein Metabolism
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Lipoprotein Metabolism
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  1. Chylomicrons
    • - Large, buoyant; apoB48
    • - Transport triglycerides from intestine (after meals)
    • - Broken down by LPL to release FFA (which are used for energy, taken up via insulin by adipose tissue, or taken up via FA-binding protein by liver to form VLDL)
    • - Becomes a Chylomicron Remnant
  2. Chylomicron Remnant
    • - Medium-sized; apoB48, apoE
    • - Results from Chylomicron degradation by LPL
    • - Taken up bythe liver
  3. VLDL
    • - Medium-sized; apoB100
    • - Transport triglycerides + some cholesterol from liver
    • - Broken down by LPL to release FFA
    • - Becomes IDL
  4. IDL
    • - Medium-sized; apoB100, ApoE
    • - Results from VLDL degradation by LPL
    • - Transport triglycerides + cholesterol
    • - Either taken up by liver or degraded by hepatic lipase to form LDL
  5. LDL
    • - Small; apoB100
    • - Transports cholesterol
    • - Internalized by LDL-R on liver & peripheral cells
    • - Atherogenic
  6. HDL
    • - Very small; apoA1
    • - Results from apoA1 binding to ABCA1 to promote efflux of cholesterol from periphreal cells
    • - Promotes reverse cholesterol transport (from periphery to liver excretion)
    • - Prevent lipoprotein oxidation
    • - Antiinflammatory effects
    • - Exchange route with triglyceride-rich lipoproteins via CETP
  7. Type I Dyslipidemia
    (Familial Chylomicronemia Syndrome)
    • - Defect in LPL
    • - Elevated chylomicrons
    • - Very elevated TG & cholesterol
    • - Increased risk of pancreatitis
    • - No increased risk of cardiovascular disease
  8. Type IIa Dyslipidemia
    (Familial Hypercholesteremia)
    • - Defect in LDL-R, apoB100
    • - Elevated LDL
    • - Very elevated cholesterol; normal TG
    • - Premature atherosclerosis & coronary disease
    • - Tendon xanthomas, eyelid xanthelasma, corneal arcus

    • - Autosomal co-dominant
    • - Can be heterozygous (LDL>500; CAD by teens)
    • - Can be homozygous (LDL~300; CAD by middle age)
  9. Type IIb Dyslipidemia
    (Familial Combined Hyperlipidemia)
    • - Unkown defect
    • - Elevated VLDL & LDL
    • - Elevated cholesterol & TG (each ~350)
    • - Premature atherosclerosis

    • - Autosomal dominant
    • - Most common inherited dyslipidemia
  10. Type III Dyslipidemia
    (Familial Dysbetalipoproteinemia)
    • - ApoE defect
    • - Elevated chylomicron remnants
    • - Elevated cholesterol & TG
    • - Premature atherosclerosis
    • - Xanthomas

    - Autosomal recessive
  11. Type IV dyslipidemia
    • - Unknown defect
    • - Very elevated TG
    • - CHD
  12. Type V Dyslipidemia
    • - Unknown defect
    • - Very elevated TG
    • - Pancreatitis, CHD
  13. Tangier's Disease
    • - ABCA1 defect
    • - Very low HDL
    • - Enlarged, orange tonsils
    • - Premature atherosclerosis

    • - Autosomal recessive
    • - Very rare

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