Turnover of apolipoprotein-B in two subjects with familial hypobetalipoproteinemia.
The metabolism of the apoprotein of low-density apoliporprotein-B was studied in a father and son with familial hypobetalipoproteinemia. The synthetic rate of apolipoprotein-B was below the normal range in both subjects, while the fractional removal rate was normal. It is concluded that the subnormal concentration of low-density lipoprotein (LDL) inthese two subjects is due to a low synthetic rate. The synthetic rate of apolipoprotein-B of very low-density lipoprotein (BLDL-apoB) measured in the father was subnormal. The fraction of the VLDL-apoB pool which was oncverted into LDL-apoB was within the normal range. It is suggested that a low synthetic rate of VLDL may underlie the disorder.
D000328:Adult; D001053:Apolipoproteins / Q000097:blood / Q000172:deficiency*; D002784:Cholesterol / Q000097:blood; D005260:Female; D006801:Humans; D007700:Kinetics; D008077:Lipoproteins, LDL / Q000097:blood / Q000172:deficiency*; D008079:Lipoproteins, VLDL / Q000097:blood; D008297:Male; D008875:Middle Aged; D010375:Pedigree
D001053:Apolipoproteins; D008077:Lipoproteins, LDL; D008079:Lipoproteins, VLDL; D002784:Cholesterol