肝性リパーゼ阻害因子がみられIII型高脂血症を呈したWeber-Christian病の一例

Abstract

Hyperlipidemia (Type III) was observed in a patient of Weber-Christian disease. Apo E-III deficiency was not observed. Post-heparin hepatic triglyceride lipase activity was low, but detectable. When the amount of post-heparin plasma was decreased to less than 20μl, the hepatic triglyceride lipase activity was higher than at more than 30μl. Inhibitory effect on hepatic triglyceride lipase was observed in intermediate lipoprotein fractions (1.017<d<1.063). Semipurified inhibitory factor inhibited triolein hydrolyzing activity of hepatic triglyceride lipase, but did not inhibited tributyrin hydrolyzing activity. These results may suggest that the inhibitor interacts with interface recognition site of hepatic triglyceride lipase, but not catalytic site.