High density lipoprotein infusion and partial plasma exchange in Tangier disease

Publisher Website
Google Scholar
Abstract
High density lipoprotein (HDL) infusion and partial plasma exchange were undertaken in 2 patients homozygous for Tangier disease. Serum samples and ultracentrifugally isolated serum fractions were analyzed over a period of 7 days post infusion by agarose electrophoresis, 2-dimensional immunoelectrophoresis (employing antibodies to HDL, HDL3, Apoprotein A-I and Apoprotein A-II), Apoprotein A radioimmunoassay, and analytical polyacrylamide electrophoresis. Immediately after HDL substitution the broad-.beta. band, normally visible upon agarose electrophoresis of Tangier plasma, resolved into a distinct .beta. and pre-.beta. band. As HDL was catabolized, an abnormal .alpha.-migrating lipoprotein was generated which contained Apoprotein A-II as protein constituent, and there was a preferential loss of Apoprotein A-I from HDL and the plasma compartment in the course of HDL catabolism. The defect in Tangier disease may be due to enhanced catabolism or defective synthesis of Apoprotein A-I.