Effect of Plasma Infusion on Plasma Apolipoprotein Levels and Lipoprotein Composition in Patients With Abetalipoproteinemia

In order to clarify the disturbance of plasma lipoprotein metabolism and the effect of supplement therapy, changes occurring in plasma lipoprotein composition and apoprotein levels after plasma infusion were studied in two sibling patients with classical abetalipoproteinemia. The patients' plasma completely lacked both apoproteins B100 and B48. After infusion of 10 ml per kilogram of body weight of normal plasma, the plasma apoprotein B level increased to 11 and 13 mg/dl and the half-life of apoprotein B to 10h and 6h respectively in the two brothers. In contrast, the plasma apoprotein E level decreased slightly or remained unchanged. On sodium dodecylsulfate polyacrylamide gel electrophoresis, apoprotein B appeared in the low-density lipoprotein fraction and the content of apoprotein E in the high-density lipoprotein fraction decreased. The mean particle size of high-density lipoprotein decreased on analysis by high-performance liquid chromatography. These results indicate that the catabolic rate of apoprotein B at low plasma levels (<15 mg/dl) is rapid in total apoprotein B deficiency, and suggest that plasma infusion may normalize apoprotein E distribution and metabolism.