Presence of two forms of apolipoprotein B in patients with dyslipoproteinemia.

Current information suggests that the major forms of the human B apolipoproteins, B-100 and B-48, are under separate genetic control and are synthesized by the liver and intestine, respectively. The apolipoprotein B composition of plasma lipoproteins has been determined in order to gain insight into the metabolic defects in patients with dyslipoproteinemias. Patients with type I and type V hyperlipoproteinemia can be separated into two groups based on apolipoprotein composition and triglyceride concentration. The first group had markedly elevated plasma triglycerides with B-48 in the 1.006 g/ml density fraction and only B-100 within IDL and LDL. The second group had plasma triglycerides less than 1200 mg % and only B-100 in all density fractions. Patients with type III hyperlipoproteinemia had B-48 in only the density less than 1.006 g/ml with B-100 in IDL and LDL; the type III hyperlipoproteinemic patient with apolipoprotein E deficiency, however, had B-48 in density less than 1.006 g/ml fraction, IDL, and LDL. Patients with type IIa, IIb, and IV hyperlipoproteinemia had only B-100 in all density fractions. These combined results are interpreted as indicating that B-48 is associated with triglyceride-rich lipoproteins synthesized by the intestine and that patients with phenotypes I, III, and V have defects in chylomicron remnant metabolism. In addition, in patients with types I and V hyperlipoproteinemia, mild hypertriglyceridemia appears to be associated with lipoprotein particles of liver origin.