出版社：American Society for Biochemistry and Molecular Biology
摘要：Lipoprotein metabolism is the result of a complex network ofmany individual components. Abnormal lipoprotein concentrationscan result from changes in the production, conversion, or catabolismof lipoprotein particles. Studies in hypolipoproteinemia andhyperlipoproteinemia have elucidated the processes that controlVLDL secretion as well as VLDL and LDL catabolism. Here, wereview the current knowledge regarding apolipoprotein B (apoB)metabolism, focusing on selected clinically relevant conditions.In hypobetalipoproteinemia attributable to truncations in apoB,the rate of secretion is closely linked to the length of apoB.On the other hand, in patients with the metabolic syndrome,it appears that substrate, in the form of free fatty acids,coupled to the state of insulin resistance can induce hypersecretionof VLDL-apoB. Studies in patients with familial hypercholesterolemia,familial defective apoB, and mutant forms of proprotein convertasesubtilisin/kexin type 9 show that mutations in the LDL receptor,the ligand for the receptor, or an intracellular chaperone forthe receptor are the most important determinants in regulatingLDL catabolism. This review also demonstrates the variance ofresults within similar, or even the same, phenotypic conditions.This underscores the sensitivity of metabolic studies to methodologicalaspects and thus the importance of the inclusion of adequatecontrols in studies.Supplementary key words very low density lipoprotein • low density lipoprotein • stable isotope • turnover study
