Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts.
作者： P. G. Pentchev , M. E. Comly , H. S. Kruth , T. Tokoro , J. Butler
DOI: 10.1096/FASEBJ.1.1.3609608
关键词: Intracellular cholesterol transport 、 LDL receptor 、 Cholesterol 、 Membrane region 、 Cholesterol storage 、 Biology 、 Low-density lipoprotein 、 Internal medicine 、 Sterol 、 Cell biology 、 Lipoprotein 、 Endocrinology
摘要: Incubation of mutant Niemann-Pick C fibroblasts with low-density lipoprotein (LDL) resulted in excessive internalization and extensive cellular over-accumulation unesterified cholesterol. The uptake LDL by the cells appeared to occur through classic receptor pathway internalized was processed lysosomes. Lipoprotein into associated delays initiation established cholesterol homeostatic responses. Subcellular fractionation accumulating LDL-cholesterol showed excess sterol be localized light lysosome-light membrane region a Percoll gradient, revealed that storage specific alteration normal profiles lysosomal marker enzymes.
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