title: High density lipoprotein infusion and partial plasma exchange in Tangier disease.
abstract: High density lipoprotein (HDL) infusion and partial plasma exchange were undertaken in two patients homozygous for Tangier disease. Serum samples and ultracentrifugally isolated serum fractions were analysed over a period of 7 days post infusion by agarose electrophoresis, two-dimensional immunoelectrophoresis (employing antibodies to HDL, HDL3, Apoprotein A-I, and Apoprotein A-II), Apoprotein A radioimmunoassay, and analytical polyacrylamide electrophoresis. The following observations were made: (a) 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; (b)as HDL was catabolized, an abnormal alpha-migrating lipoprotein was generated which contained Apoprotein A-II as protein constituent; and (c) there was a proferential loss of Apoprotein A-I from HDL and the plasma compartment in the course of HDL catabolism. The results suggest that the defect in Tangier disease resides with enhanced catabolism or defective synthesis of Apoprotein A-I.
mesh_terms: D001053:Apolipoproteins / Q000097:blood; D001803:Blood Transfusion; D002784:Cholesterol / Q000097:blood; D004587:Electrophoresis, Agar Gel; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D007009:Hypolipoproteinemias / Q000097:blood*; D007122:Immunoelectrophoresis; D008075:Lipoproteins, HDL / Q000008:administration & dosage* / Q000097:blood; D013631:Tangier Disease / Q000097:blood*
chemical_list: D001053:Apolipoproteins; D008075:Lipoproteins, HDL; D002784:Cholesterol