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Exogenous
cycle Endogenous
cycle
Metabolism of chylomicrons.
CM=chylomicron; TAG=triacylglycerol; C=cholesterol; CE=cholesterol
esters
Reference : Lippincott
Metabolism of VLDL & LDL
TAG=triacylglycerol; VLDL=very low density lipoprotein; LDL=low
density lipoprotein; C=cholesterol; CE=cholesterol
Reference : Lippincott
esters
Endogenous cycle
HDL-cholestrol metabolism
CETP
VLDL
Antioxidants Free radicals
+
-
Scavenger receptor
Unregulated mechanism for LDL
Hyperlipoproteinaemias
Metabolic defect
1. Type-I
(Familial Lipoprotein
lipase deficeency)
๏€ฃTG
๏€ฃ Chylomicrone
๏€ฃ VLDL
๏‚ฏ HDL & LDL
Autosomal
recessive
Lipoprotein lipase
2. Type-II
(Familial Hyper-
cholestrolaemia) (FHC)
๏€ฃLDL
๏€ฃ T. cholesterol
๏€ฃ VLDL & TGs
Autosomal
dominant
โ€ข Metabolic defect.
โ€ข Defect LDL catabolism
3. Type-III
(Familial dys-beta
lipoproteinaemia)
๏€ฃLDL
๏€ฃ VLDL
๏€ฃ T. cholesterol & TGs
Autosomal
dominant
โ€ข Metabolic defect
โ€ข Apo-E
โ€ข Defect conversion of VLDL to
IDL
4. Type-IV
(Familial
Hypertriglyceridaemia)
(FHTG)
๏€ฃVLDL
๏€ฃ TG synthesis
Cholesterol normal/๏€ฃ
๏‚ฏ HDL, ๏‚ฏ LDL
Autosomal
dominant
โ€ข Metabolic defect
โ€ข ๏€ฃ Tg synthesis
โ€ข ๏‚ฏ Catabolism of Tg & cholesterol
โ€ข Glucose intolerence
5. Type-V
(Combined
Hyperlipidaemia)
๏€ฃChylomicrone & VLDL
๏€ฃ TGs & ๏€ฃ Cholesterol
๏‚ฏ HDL & LDL
Autosomal
dominant
-
Hypolipoproteinaemia
Clinical feature Metabolic defect
1.๏ข-
Lipoproteinaemia
(Bassen Kornzweig
syndrome)
๏‚ฏCholesterol
(Absence of
LDL)
Low Tg conc.
No
chylomicrone
No VLDL
โ€ข Red blood cell
abnormalities.
โ€ข Malabsorption of
fat.
โ€ข Fatty infiltration.
Synthesis of
Apo-B
2. ๏ก-Lipoprotein
Deficiency
๏‚ฏHDL or
absent
โ€ข Hyperplastic
orange
yellow tonsils
๏‚ฏApo-I, II
Accumulation of
cholesterol
esters in
different tissu.
Palmar xanthoma
Orange yellow tonsil : Tangier disease
5 lipoprotein metabolism.ppt

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5 lipoprotein metabolism.ppt

  • 1.
  • 3.
  • 4. Metabolism of chylomicrons. CM=chylomicron; TAG=triacylglycerol; C=cholesterol; CE=cholesterol esters Reference : Lippincott
  • 5. Metabolism of VLDL & LDL TAG=triacylglycerol; VLDL=very low density lipoprotein; LDL=low density lipoprotein; C=cholesterol; CE=cholesterol Reference : Lippincott esters
  • 7.
  • 8.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14.
  • 15. Antioxidants Free radicals + - Scavenger receptor Unregulated mechanism for LDL
  • 16.
  • 17. Hyperlipoproteinaemias Metabolic defect 1. Type-I (Familial Lipoprotein lipase deficeency) ๏€ฃTG ๏€ฃ Chylomicrone ๏€ฃ VLDL ๏‚ฏ HDL & LDL Autosomal recessive Lipoprotein lipase 2. Type-II (Familial Hyper- cholestrolaemia) (FHC) ๏€ฃLDL ๏€ฃ T. cholesterol ๏€ฃ VLDL & TGs Autosomal dominant โ€ข Metabolic defect. โ€ข Defect LDL catabolism 3. Type-III (Familial dys-beta lipoproteinaemia) ๏€ฃLDL ๏€ฃ VLDL ๏€ฃ T. cholesterol & TGs Autosomal dominant โ€ข Metabolic defect โ€ข Apo-E โ€ข Defect conversion of VLDL to IDL 4. Type-IV (Familial Hypertriglyceridaemia) (FHTG) ๏€ฃVLDL ๏€ฃ TG synthesis Cholesterol normal/๏€ฃ ๏‚ฏ HDL, ๏‚ฏ LDL Autosomal dominant โ€ข Metabolic defect โ€ข ๏€ฃ Tg synthesis โ€ข ๏‚ฏ Catabolism of Tg & cholesterol โ€ข Glucose intolerence 5. Type-V (Combined Hyperlipidaemia) ๏€ฃChylomicrone & VLDL ๏€ฃ TGs & ๏€ฃ Cholesterol ๏‚ฏ HDL & LDL Autosomal dominant -
  • 18. Hypolipoproteinaemia Clinical feature Metabolic defect 1.๏ข- Lipoproteinaemia (Bassen Kornzweig syndrome) ๏‚ฏCholesterol (Absence of LDL) Low Tg conc. No chylomicrone No VLDL โ€ข Red blood cell abnormalities. โ€ข Malabsorption of fat. โ€ข Fatty infiltration. Synthesis of Apo-B 2. ๏ก-Lipoprotein Deficiency ๏‚ฏHDL or absent โ€ข Hyperplastic orange yellow tonsils ๏‚ฏApo-I, II Accumulation of cholesterol esters in different tissu.
  • 20.
  • 21. Orange yellow tonsil : Tangier disease

Editor's Notes

  1. Control of plasma LDL production and uptake by liver LDL receptors. (a) In normal human subjects, VLDL is secreted by the liver and converted to IDL in the capillaries of the peripheral tissues. About half of the plasma IDL particles bind to the LDL receptor and are taken up by the liver.The remainder are converted to LDL at the peripheral tissues.
  2. (c) In normal individuals who have a long-term high-cholesterol diet, the liver is filled with cholesterol, which represses the rate of LDL receptor production. Receptor deficiency, whether of genetic or dietary cause, raises the plasma LDL level by increasing the rate of LDL production and decreasing the rate of LDL uptake.
  3. (b) In individuals with familial hypercholesterolemia (FH), liver LDL receptors are diminished or eliminated because of a genetic
  4. Fredricksonโ€™s classification to hyperlipoproteinmias is very important because: 1) They focus on different groups of metabolic abnormalities related to hyperlipoproteinmias. 2) Associated different lipoprotein types with certain clinical features. 3) Provide basis for diet & drug therapy. Lipoprotein disorders may be classified also as: 1- Primary disorder : a) genetic (familial) b- non-genetic (sporadic). 2- Secondary disorder : arise from another cause eg. Diet , alcohol, drugs, or disease of metabolic cause a) hormonal (hypothyroidism , diabetes ) or b) malignant or infectious.