NDLI: Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

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Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

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Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Watari, H. Glick, J. M. Dwyer, N. K. Blanchette-mackie, E. J. Neufeld, E. B. Pentchev, P. G. Patel, S. Brady, R. O. Strauss, J. F.
Description	Author Affiliation: Watari H ( Center for Research on Reproduction and Women's Health, Departments of Obstetrics and Gynecology, University of Pennsylvania Medical Center, Philadelphia, PA 19104, USA.);
Abstract	Niemann-Pick type C (NPC) disease is an inherited lipid storage disorder that affects the viscera and central nervous system. A characteristic feature of NPC cells is the lysosomal accumulation of low density lipoprotein-derived cholesterol. To elucidate important structural features of the recently identified NPC1 gene product defective in NPC disease, we examined the ability of wild-type NPC1 and NPC1 mutants to correct the excessive lysosomal storage of low density lipoprotein-derived cholesterol in a model cell line displaying the NPC cholesterol-trafficking defect (CT60 Chinese hamster ovary cells). CT60 cells transfected with human wild-type NPC1 contained immunoreactive proteins of 170 and 190 kDa localized to the lysosomal/endosomal compartment. Wild-type NPC1 protein corrected the NPC cholesterol-trafficking defect in the CT60 cells. Mutation of conserved cysteine residues in the NPC1 N terminus to serine residues resulted in proteins targeted to lysosomal membranes encircling cholesterol-laden cores, whereas deletion of the C-terminal 4-aa residues containing the LLNF lysosome-targeting motif resulted in the expression of protein localized to the endoplasmic reticulum. None of these mutant NPC1 proteins corrected the NPC cholesterol-trafficking defect in CT60 cells. We conclude that transport of the NPC1 protein to the cholesterol-laden lysosomal compartment is essential for expression of its biological activity and that domains in the N terminus of the NPC1 protein are critical for mobilization of cholesterol from lysosomes.
ISSN	00278424
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	3
Volume Number	96
Language	English
Publisher	National Academy of Sciences
Publisher Date	1999-03-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Carrier Proteins Cholesterol Metabolism Lysosomes Membrane Glycoproteins Proteins Amino Acid Substitution Animals CHO Cells Cricetinae Endoplasmic Reticulum Filipin Green Fluorescent Proteins Luminescent Proteins Mutagenesis, Site-Directed Niemann-Pick Diseases Genetics Chemistry Recombinant Proteins Sequence Deletion Transfection Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Multidisciplinary
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

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