The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Group C Niemann‐Pick disease: faulty regulation of low‐density lipoprotein uptake and cholesterol storage in cultured fibroblasts
Abstract
Incubation of mutant Niemann‐Pick C fibroblasts with low‐density lipoprotein (LDL) resulted in excessive internalization of lipoprotein and extensive cellular over‐accumulation of unesterified cholesterol. The uptake of LDL by the mutant cells appeared to occur through the classic LDL receptor pathway and internalized lipoprotein was processed in lysosomes. Lipoprotein uptake into mutant cells was associated with delays in the initiation of established cellular cholesterol homeostatic responses. Subcellular fractionation of mutant Niemann‐Pick C fibroblasts accumulating LDL‐cholesterol showed excess unesterified sterol to be localized in the light lysosome‐light membrane region of a Percoll gradient, and revealed that cholesterol storage was associated with a specific alteration in the normal profiles of lysosomal marker enzymes.— P entchev , P. G.; C omly , M. E.; K ruth , H. S.; T okoro , T; B utler , J.; S okol , J.; F illing ‐K atz , M.; Q uirk , J. M.; M arshall , D. C.; P atel , S.; V anier , M. T.; B rady , R. O. Group C Nieman‐Pick disease: faulty regulation of low‐density lipoprotein uptake and cholesterol storage in cultured fibroblasts. FASEB J. 1: 40‐45; 1987.
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Citations
Cites
A Receptor-Mediated Pathway for Cholesterol Homeostasis
Brown, M. S., & Goldstein, J. L. (1986) · Science · doi:10.1126/science.3513311 · Cited by 5,703
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Regulation of the mevalonate pathway
Goldstein, J. L., & Brown, M. S. (1990) · Nature · doi:10.1038/343425a0 · Cited by 5,394
Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition) 1
Klionsky, D. J., Abdel-Aziz, A. K., Abdelfatah, S., Abdellatif, M., Abdoli, A., Abel, S., Abeliovich, H., Abildgaard, M. H., Abudu, Y. P., Acevedo-Arozena, A., et al. (2021) · Autophagy · doi:10.1080/15548627.2020.1797280 · Cited by 2,897
Niemann-Pick C1 Disease Gene: Homology to Mediators of Cholesterol Homeostasis
Carstea, E. D., Morris, J. A., Coleman, K. G., Loftus, S. K., Zhang, D., Cummings, C., Gu, J., Rosenfeld, M. A., Pavan, W. J., Krizman, D. B., et al. (1997) · Science · doi:10.1126/science.277.5323.228 · Cited by 1,293
Self-citationVanier, M. T. (2010) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-5-16 · Cited by 1,222
Self-citationMiglustat for treatment of Niemann-Pick C disease: a randomised controlled study
Patterson, M. C., Vecchio, D., Prady, H., Abel, L., & Wraith, J. E. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70194-1 · Cited by 608
Metadata
- Authors
- Pentchev, P. G., Comly, M. E., Kruth, H. S., Tokoro, T., Butler, J., Sokol, J., Filling‐Katz, M., Quirk, J. M., Marshall, D. C., Patel, S., Vanier, M. T., & Brady, R. O.
- Venue
- The FASEB Journal
- Publisher
- Wiley
- Published
- 1987-07
- Volume
- 1
- Issue
- 1
- Pages
- 40-45
- ISSN
- 0892-6638 (ISSN-L)
- 1530-6860
- OpenAlex
- W1517698792
- Cited by
- 195
- References
- 1
- FWCI
- 8.65
- Type
- journal article
- Language
- en
- Open access
- closed
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- The paper
- Pentchev, P. G., Comly, M. E., Kruth, H. S., Tokoro, T., Butler, J., Sokol, J., Filling‐Katz, M., Quirk, J. M., Marshall, D. C., Patel, S., Vanier, M. T., & Brady, R. O. (1987). Group C Niemann‐Pick disease: faulty regulation of low‐density lipoprotein uptake and cholesterol storage in cultured fibroblasts. The FASEB Journal. https://doi.org/10.1096/fasebj.1.1.3609608
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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.
