| pubmed-article:18591242 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:18591242 | lifeskim:mentions | umls-concept:C0002508 | lld:lifeskim |
| pubmed-article:18591242 | lifeskim:mentions | umls-concept:C0542341 | lld:lifeskim |
| pubmed-article:18591242 | lifeskim:mentions | umls-concept:C1550605 | lld:lifeskim |
| pubmed-article:18591242 | lifeskim:mentions | umls-concept:C0456205 | lld:lifeskim |
| pubmed-article:18591242 | lifeskim:mentions | umls-concept:C1998811 | lld:lifeskim |
| pubmed-article:18591242 | pubmed:issue | 36 | lld:pubmed |
| pubmed-article:18591242 | pubmed:dateCreated | 2008-9-1 | lld:pubmed |
| pubmed-article:18591242 | pubmed:abstractText | Mutations in the late endosomal/lysosomal membrane protein Niemann-Pick C1 (NPC1) are known to cause a generalized block in retrograde vesicle-mediated transport, resulting in the hyper-accumulation of multiple lysosomal cargos. An important, yet often overlooked, category of lysosomal cargo includes the vast array of small molecular weight amine-containing molecules that are substrates for ion trapping in the highly acidic organelle lumen. We show here that the introduction of amine-containing molecules in lysosomes can significantly stimulate NPC1-mediated late endosome/lysosome fusion, and subsequently the secretion of lysosomal cargo. To illustrate the physiological importance of this NPC1-mediated transport pathway, we show that NPC1-deficient cells are more susceptible to the toxic effects of a lysosomotropic polyamine metabolite 3-aminopropanal. Moreover, NPC fibroblasts are shown to have higher levels of polyamine oxidase, an enzyme involved in the formation of 3-aminopropanal. Collectively, these findings provide strong support for a novel functional role for NPC1 and may also provide clues toward understanding NPC disease progression. | lld:pubmed |
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| pubmed-article:18591242 | pubmed:language | eng | lld:pubmed |
| pubmed-article:18591242 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:18591242 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:18591242 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:18591242 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:18591242 | pubmed:month | Sep | lld:pubmed |
| pubmed-article:18591242 | pubmed:issn | 0021-9258 | lld:pubmed |
| pubmed-article:18591242 | pubmed:author | pubmed-author:KriseJeffrey... | lld:pubmed |
| pubmed-article:18591242 | pubmed:author | pubmed-author:KaufmannAllyn... | lld:pubmed |
| pubmed-article:18591242 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:18591242 | pubmed:day | 5 | lld:pubmed |
| pubmed-article:18591242 | pubmed:volume | 283 | lld:pubmed |
| pubmed-article:18591242 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:18591242 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:18591242 | pubmed:pagination | 24584-93 | lld:pubmed |
| pubmed-article:18591242 | pubmed:dateRevised | 2011-11-2 | lld:pubmed |
| pubmed-article:18591242 | pubmed:meshHeading | pubmed-meshheading:18591242... | lld:pubmed |
| pubmed-article:18591242 | pubmed:meshHeading | pubmed-meshheading:18591242... | lld:pubmed |
