The DOI matches its Crossref record; 62 of the 62 references it cites were found in a registry or an index.
Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells
Open accessAbstract
We recently showed that human skin fibroblasts internalize fluorescent analogues of the glycosphingolipids lactosylceramide and globoside almost exclusively by a clathrin-independent mechanism involving caveolae.In contrast, a sphingomyelin analogue is internalized approximately equally via clathrin-dependent and caveolar routes.Here, we further characterized the caveolar pathway for glycosphingolipids, showing that Golgi targeting of sphingolipids internalized via caveolae required microtubules and phosphoinositol 3-kinases and was inhibited in cells expressing dominant-negative Rab7 and Rab9 constructs.In addition, overexpression of wild-type Rab7 or Rab9 (but not Rab11) in Niemann-Pick type C (NP-C) lipid storage disease fibroblasts resulted in correction of lipid trafficking defects, including restoration of Golgi targeting of fluorescent lactosylceramide and endogenous GM 1 ganglioside, and a dramatic reduction in intracellular cholesterol stores.Our results demonstrate a role for Rab7 and Rab9 in the Golgi targeting of glycosphingolipids and suggest a new therapeutic approach for restoring normal lipid trafficking in NP-C cells.
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Cites
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The position of lysosomes within the cell determines their luminal pH
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Metadata
- Authors
- Choudhury, A., Dominguez, M., Puri, V., Sharma, D. K., Narita, K., Wheatley, C. L., Marks, D. L., & Pagano, R. E.
- Venue
- Journal of Clinical Investigation
- Published
- 2002-06-15
- Volume
- 109
- Issue
- 12
- Pages
- 1541-1550
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W4238609105
- Cited by
- 380
- References
- 62
- FWCI
- 10.84
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Choudhury, A., Dominguez, M., Puri, V., Sharma, D. K., Narita, K., Wheatley, C. L., Marks, D. L., & Pagano, R. E. (2002). Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. Journal of Clinical Investigation. https://doi.org/10.1172/jci0215420
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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