Mechanisms of hypercholesterolaemia in glycogen storage disease type I: defective metabolism of low density lipoprotein in cultured skin fibroblasts.
作者： E. LEVY , L. THIBAULT , C. C. ROY , J. LETARTE , M. LAMBERT
DOI: 10.1111/J.1365-2362.1990.TB01852.X
关键词:
摘要: Hyperlipidaemia is a feature of glycogen storage disease type I (GSD-I) (Levy et al.). High levels LDL cholesterol (200 +/- 25 mg dl-1) and apo B (387 44 were found in association with hypercholesterolaemia GSD-I. Related causative factors might be attributed to overproduction and/or delayed removal LDL. In this study, possible alteration the clearance was examined. Using cultured fibroblasts for receptor activity, following observations made: 1. GSD-I revealed only slight decrease binding (65 7) when compared controls (74 4 ng mg-1 protein), however, internalization (382 24 vs. 570 52 protein) proteolytic degradation (2082 280 2916 12.5 significantly affected (P less than 0.01). 2. Binding, from that controls, lower 3. Substitution control lipoprotein-deficient serum (LPDS) by LPDS further diminished above processes 0.05). Our results demonstrate increased plasma due decreased catabolism The data suggest problem may well multifactorial, expression, abnormal composition impaired interaction circulating inhibitory factor.
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