Please use this identifier to cite or link to this item: http://hdl.handle.net/2381/45537
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dc.contributor.authorWheeler, Simon-
dc.contributor.authorSchmid, Ralf-
dc.contributor.authorSillence, Dan J.-
dc.date.accessioned2019-09-10T13:17:05Z-
dc.date.available2019-09-10T13:17:05Z-
dc.date.issued2019-02-07-
dc.identifier.citationInternational Journal of Molecular Sciences, 2019, 20(3), 717;en
dc.identifier.urihttps://www.mdpi.com/1422-0067/20/3/717en
dc.identifier.urihttp://hdl.handle.net/2381/45537-
dc.descriptionSupplementary materials can be found at http://www.mdpi.com/1422-0067/20/3/717/s1.en
dc.description.abstractThe accumulation of lipids in the late endosomes and lysosomes of Niemann⁻Pick type C disease (NPCD) cells is a consequence of the dysfunction of one protein (usually NPC1) but induces dysfunction in many proteins. We used molecular docking to propose (a) that NPC1 exports not just cholesterol, but also sphingosine, (b) that the cholesterol sensitivity of big potassium channel (BK) can be traced to a previously unappreciated site on the channel's voltage sensor, (c) that transient receptor potential mucolipin 1 (TRPML1) inhibition by sphingomyelin is likely an indirect effect, and (d) that phosphoinositides are responsible for both the mislocalization of annexin A2 (AnxA2) and a soluble NSF (N-ethylmaleimide Sensitive Fusion) protein attachment receptor (SNARE) recycling defect. These results are set in the context of existing knowledge of NPCD to sketch an account of the endolysosomal pathology key to this disease.en
dc.language.isoenen
dc.publisherMDPIen
dc.relation.urihttps://www.ncbi.nlm.nih.gov/pubmed/30736449-
dc.rightsCopyright © the authors, 2019. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.en
dc.subjectAnnexin A2en
dc.subjectBKen
dc.subjectNPC1en
dc.subjectNiemann–Picken
dc.subjectSNAREen
dc.subjectTRPML1en
dc.subjectlipidsen
dc.titleLipid–Protein Interactions in Niemann–Pick Type C Disease: Insights from Molecular Modelingen
dc.typeJournal Articleen
dc.identifier.doi10.3390/ijms20030717-
dc.identifier.eissn1422-0067-
dc.identifier.piiijms20030717-
dc.description.statusPeer-revieweden
dc.description.versionPublisher Versionen
dc.type.subtypeJournal Article-
pubs.organisational-group/Organisationen
pubs.organisational-group/Organisation/COLLEGE OF LIFE SCIENCESen
pubs.organisational-group/Organisation/COLLEGE OF LIFE SCIENCES/Biological Sciencesen
pubs.organisational-group/Organisation/COLLEGE OF LIFE SCIENCES/Biological Sciences/Molecular & Cell Biologyen
pubs.organisational-group/Organisation/COLLEGE OF LIFE SCIENCES/Themesen
pubs.organisational-group/Organisation/COLLEGE OF LIFE SCIENCES/Themes/Molecular & Cellular Bioscienceen
dc.dateaccepted2019-02-03-
Appears in Collections:Published Articles, Dept. of Molecular and Cell Biology

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