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Cited by 373

The DOI matches its Crossref record; 46 of the 46 references it cites were found in a registry or an index.

The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo

Open access
Neufeld, Edward B., Wastney, Meryl, Patel, Shutish, Suresh, Sundar, Cooney, Adele M., Dwyer, Nancy K., Roff, Calvin F., Ohno, Kousaku, Morris, Jill A., Carstea, Eugene D., Incardona, John P., Strauss, Jerome F., Vanier, Marie T., Patterson, Marc C., Brady, Roscoe O., Pentchev, Peter G., Blanchette-Mackie, E. Joan·1999·Journal of Biological Chemistry
DOI 10.1074/jbc.274.14.9627
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Abstract

Niemann-Pick C disease (NP-C) is a neurovisceral lysosomal storage disorder. A variety of studies have highlighted defective sterol trafficking from lysosomes in NP-C cells. However, the heterogeneous nature of additional accumulating metabolites suggests that the cellular lesion may involve a more generalized block in retrograde lysosomal trafficking. Immunocytochemical studies in fibroblasts reveal that the NPC1 gene product resides in a novel set of lysosome-associated membrane protein-2 (LAMP2)(+)/mannose 6-phosphate receptor(-) vesicles that can be distinguished from cholesterol-enriched LAMP2(+) lysosomes. Drugs that block sterol transport out of lysosomes also redistribute NPC1 to cholesterol-laden lysosomes. Sterol relocation from lysosomes in cultured human fibroblasts can be blocked at 21 degrees C, consistent with vesicle-mediated transfer. These findings suggest that NPC1(+) vesicles may transiently interact with lysosomes to facilitate sterol relocation. Independent of defective sterol trafficking, NP-C fibroblasts are also deficient in vesicle-mediated clearance of endocytosed [14C]sucrose. Compartmental modeling of the observed [14C]sucrose clearance data targets the trafficking defect caused by mutations in NPC1 to an endocytic compartment proximal to lysosomes. Low density lipoprotein uptake by normal cells retards retrograde transport of [14C]sucrose through this same kinetic compartment, further suggesting that it may contain the sterol-sensing NPC1 protein. We conclude that a distinctive organelle containing NPC1 mediates retrograde lysosomal transport of endocytosed cargo that is not restricted to sterol.

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Metadata

Authors
Neufeld, E. B., Wastney, M., Patel, S., Suresh, S., Cooney, A. M., Dwyer, N. K., Roff, C. F., Ohno, K., Morris, J. A., Carstea, E. D., Incardona, J. P., Strauss, J. F., Vanier, M. T., Patterson, M. C., Brady, R. O., Pentchev, P. G., & Blanchette-Mackie, E. J.
Venue
Journal of Biological Chemistry
Publisher
Elsevier BV
Published
1999-04
Volume
274
Issue
14
Pages
9627-9635
ISSN
0021-9258 (ISSN-L)
DOI
10.1074/jbc.274.14.9627
OpenAlex
W2096221379
Cited by
373
References
46
FWCI
20.24
Type
journal article
Language
en
Open access
hybrid

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CoChat. (2026). Citation check: The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo. https://app.cochat.ai/papers/10.1074/jbc.274.14.9627
The paper
Neufeld, E. B., Wastney, M., Patel, S., Suresh, S., Cooney, A. M., Dwyer, N. K., Roff, C. F., Ohno, K., Morris, J. A., Carstea, E. D., Incardona, J. P., Strauss, J. F., Vanier, M. T., Patterson, M. C., Brady, R. O., Pentchev, P. G., & Blanchette-Mackie, E. J. (1999). The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.274.14.9627

Data sources

Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.