{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T14:42:08Z","timestamp":1790779328255,"version":"4.1.0"},"reference-count":60,"publisher":"The Company of Biologists","issue":"5","license":[{"start":{"date-parts":[[2023,3,2]],"date-time":"2023-03-02T00:00:00Z","timestamp":1677715200000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100015411","name":"Ara Parseghian Medical Research Fund","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100015411","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Niemann-Pick Canada"},{"DOI":"10.13039\/100008109","name":"University of Notre Dame","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100008109","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100008109","name":"University of Notre Dame","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100008109","id-type":"DOI","asserted-by":"crossref"}]},{"name":"National Science Foundation"},{"name":"National Science Foundation"},{"DOI":"10.13039\/100008109","name":"University of Notre Dame","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100008109","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100005848","name":"Henry Luce Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100005848","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,3,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>The pathological accumulation of cholesterol is a signature feature of Niemann\u2013Pick type C (NPC) disease, in which excessive lipid levels induce Purkinje cell death in the cerebellum. NPC1 encodes a lysosomal cholesterol-binding protein, and mutations in NPC1 drive cholesterol accumulation in late endosomes and lysosomes (LE\/Ls). However, the fundamental role of NPC proteins in LE\/L cholesterol transport remains unclear. Here, we demonstrate that NPC1 mutations impair the projection of cholesterol-containing membrane tubules from the surface of LE\/Ls. A proteomic survey of purified LE\/Ls identified StARD9 as a novel lysosomal kinesin responsible for LE\/L tubulation. StARD9 contains an N-terminal kinesin domain, a C-terminal StART domain, and a dileucine signal shared with other lysosome-associated membrane proteins. Depletion of StARD9 disrupts LE\/L tubulation, paralyzes bidirectional LE\/L motility and induces accumulation of cholesterol in LE\/Ls. Finally, a novel StARD9 knock-out mouse recapitulates the progressive loss of Purkinje cells in the cerebellum. Together, these studies identify StARD9 as a microtubule motor protein responsible for LE\/L tubulation and provide support for a novel model of LE\/L cholesterol transport that becomes impaired in NPC disease.<\/jats:p>","DOI":"10.1242\/jcs.260662","type":"journal-article","created":{"date-parts":[[2023,3,2]],"date-time":"2023-03-02T11:55:30Z","timestamp":1677758130000},"source":"Crossref","is-referenced-by-count":3,"title":["StARD9 is a novel lysosomal kinesin required for membrane tubulation, cholesterol transport and Purkinje cell survival"],"prefix":"10.1242","volume":"136","author":[{"given":"Felicity R.","family":"Sterling","sequence":"first","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jon","family":"D'Amico","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandria M.","family":"Brumfield","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kara L.","family":"Huegel","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia S.","family":"Vaughan","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Morris","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , 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USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew M.","family":"Champion","sequence":"additional","affiliation":[{"name":"University of Notre Dame 2 Department of Chemistry and Biochemistry , , Notre Dame, IN 46556 , USA"},{"name":"University of Notre Dame Proteomics and Mass Spectrometry Facility, University of Notre Dame 3 , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Maciuba","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Allen","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Marasco","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grant","family":"Koch","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Gonzalez","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon","family":"Hodges","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon","family":"Leahy","sequence":"additional","affiliation":[{"name":"University of Notre Dame 1 Department of Biological Sciences , , Notre Dame, IN 46556 , 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