lipid metabolism Flashcards
what is familial chylomicronemia
it is familial hyperlipoproteinemia type 1 syndrome
defects in LPL enz and / or ApoC-II
how is lipoprotein lipase activity measured
normal LPL remains attached to heparan sulfate of endothelial cells. whereby the act on circulating VLDL and chylomicrons to release FFA in adjacent tissues.
In order to measures LPL levels -IV heparin is administered which releases the LPL– if the activity is low there is LPL deficiency
name the inherited hyper- lipoproteinemias
- Familial chylomicronemia
- Familial hypercholesterolemia
- Familial dysbeta-lipoproteinemia
- Familial hyper-triglyceridemia
Protein defective in familial chylomicronemia
Elevated lipoprotein in Chylomicronemia
Lipoprotein lipase deficiency and ApoC-II are deficient
Chylomicrons are elevated
Protein defective in familial hypercholesterolemia
Elevated lipoprotein in hypercholesterolemia
LDL receptor and its ligand ApoB-100 are defective
LDL is elevated.
Protein defective in familial dysbeta lipoproteinemia
Elevated lipoprotein in dysbeta lipoproteinemia
Apo E is defective
Chylomicrons and VLDL remnants are elevated.
Protein defective in familial hypertriglyceridemia
Elevated lipoprotein in hypertriglyceridemia
Apo A-V are elevated.
VLDL is elevated.
hypertriglyceridemia is a risk for which conditions
pancreatitis
obesity and insulin resistance
Premature coronary artery disease is seen with which inherited hyperlipoproteinemia
hypercholesterolemia and dysbeta- lipoproteinemia
with elevated LDL and Chylomicron/ VLDL remnants.
acute pancreatitis is seen as a risk in which inherited lipoproteinemia
familial hyperchylomicronemia
familial hypertriglyceridemia
tendon xanthomas and eyelid xanthelasmas are seen only in
hypercholesterolemia
