Niemann-Pick disease type C could result from a genetic deficiency of cholesterol egress from the lysosomes.

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作者信息

Den Tandt W R, van Hoof F

机构信息

Faculty of Medicine, University of Antwerp (UIA), Belgium.

出版信息

J Inherit Metab Dis. 1989;12(4):483-4. doi: 10.1007/BF01802046.

DOI:10.1007/BF01802046

PMID:2516179

Abstract

摘要

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本文引用的文献

Type C Niemann-Pick disease. A parallel loss of regulatory responses in both the uptake and esterification of low density lipoprotein-derived cholesterol in cultured fibroblasts.

J Biol Chem. 1986 Dec 15;261(35):16775-80.

Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol.

J Biol Chem. 1988 Mar 5;263(7):3411-7.

Type-C Niemann-Pick disease: low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes.

Proc Natl Acad Sci U S A. 1988 Nov;85(21):8022-6. doi: 10.1073/pnas.85.21.8022.

Role of acylcoenzyme A: cholesterol o-acyltransferase in cholesterol metabolism.

Prog Lipid Res. 1979;18(1):31-53. doi: 10.1016/0163-7827(79)90003-1.

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