{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T07:53:17Z","timestamp":1775893997213,"version":"3.50.1"},"reference-count":46,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2020,4,27]],"date-time":"2020-04-27T00:00:00Z","timestamp":1587945600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:p>Niemann\u2010Pick disease type C (NPC) is a neurovisceral lipid\u2010storage disease. Although NPC patients show lipid storage in anterior horn cells of the spinal cord, little information is available regarding the electron microscopic analyses of the morphologies of intra\u2010endosomal lipid like\u2010materials in the anterior horn cells of NPC patients. In this study, we elucidated the intra\u2010endosomal ultrastructures in spinal anterior horn cells in an NPC patient, as well as in mutant BALB\/c NPC1<jats:sup>\u2212\/\u2212<\/jats:sup> mice with a retroposon insertion in the <jats:italic>NPC1<\/jats:italic> gene. These morphologies were classified into four types: vesicle, multiple concentric sphere (MCS), membrane, and rose flower. The percentages of the composition in the NPC patient and NPC1<jats:sup>\u2212\/\u2212<\/jats:sup> mice were: vesicle (55.5% and 14.9%), MCS (15.7% and 3.4%), membrane (23.6% and 57.1%), and rose flower (5.2% and 24.6%), respectively. Formation of the intra\u2010endosomal structures could proceed as follows: (i) a vesicle or MCS buds off the endosome into the lumen; (ii) when a vesicle breaks down, a membrane is formed; and (iii) after an MCS breaks down, a rose flower structure is formed. Our new finding in this study is that ultrastructural morphology is the same between the NPC patient and NPC1<jats:sup>\u2212\/\u2212<\/jats:sup> mice, although there are differences in the composition.<\/jats:p>","DOI":"10.1111\/pin.12934","type":"journal-article","created":{"date-parts":[[2020,4,28]],"date-time":"2020-04-28T04:11:20Z","timestamp":1588047080000},"page":"422-432","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Ultrastructure of spinal anterior horn cells in human Niemann\u2010Pick type C (NPC) patient and mouse model of NPC with retroposon insertion in NPC1 genes"],"prefix":"10.1111","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5158-1568","authenticated-orcid":false,"given":"Masahiro","family":"Kusunoki\u2010Ii","sequence":"first","affiliation":[{"name":"Division of Neuropathology, Faculty of Medicine Tottori University  Tottori 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