Reversible abnormalities of low density lipoprotein composition in familial hypercholesterolaemia.
Low density lipoprotein (LDL) composition was analysed in twenty-one patients with familial hypercholesterolaemia, including six homozygotes. By comparison with nineteen controls the patients' LDL had an increased ratio of cholesterol:phospholipid and a decreased ratio of lecithin:sphingomyelin; these changes were more marked in homozygotes than in heterozygotes. Treatment of sevel patients with plasma exchange temporarily resulted in near-normalization of the composition of their LDL. These results suggest that abnormalities of LDL composition in familial hypercholesterolaemia are secondary to hypocatabolism of LDL, which prolongs the half-life of LDL and thus increases the mean age of the population of particles circulating in plasma.
D001803:Blood Transfusion; D002784:Cholesterol / Q000032:analysis; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D006937:Hypercholesterolemia / Q000097:blood / Q000235:genetics* / Q000628:therapy; D008077:Lipoproteins, LDL / Q000378:metabolism*; D008297:Male; D010713:Phosphatidylcholines / Q000032:analysis; D010743:Phospholipids / Q000032:analysis; D010949:Plasma
D008077:Lipoproteins, LDL; D010713:Phosphatidylcholines; D010743:Phospholipids; D002784:Cholesterol