{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T06:51:23Z","timestamp":1790664683936,"version":"4.1.0"},"reference-count":68,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T00:00:00Z","timestamp":1541980800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Introduction: Olfactory impairment is one of the earliest symptoms in neurodegenerative disorders that has also been documented in Niemann-Pick disease type C1 (NPC1). NPC1 is a very rare, neurovisceral lipid storage disorder, characterized by a deficiency of Npc1 gene function that leads to progressive neurodegeneration. Here, we compared the pathologic effect of defective Npc1 gene on the vomeronasal neuroepithelium (VNE) with that of the olfactory epithelium (OE) in an NPC1 mouse model. Methods: Proliferation in the VNE and OE was assessed by applying a bromodeoxyuridine (BrdU) protocol. We further compared the immunoreactivities of anti-olfactory marker protein (OMP), and the lysosomal marker cathepsin-D in both epithelia. To investigate if degenerative effects of both olfactory systems can be prevented or reversed, some animals were treated with a combination of miglustat\/allopregnanolone\/2-hydroxypropyl-cyclodextrin (HP\u03b2CD), or a monotherapy with HP\u03b2CD alone. Results: Using BrdU to label dividing cells of the VNE, we detected a proliferation increase of 215% \u00b1 12% in Npc1\u2212\/\u2212 mice, and 270% \u00b1 10% in combination- treated Npc1\u2212\/\u2212 animals. The monotherapy with HP\u03b2CD led to an increase of 261% \u00b1 10.5% compared to sham-treated Npc1\u2212\/\u2212 mice. Similar to the OE, we assessed the high regenerative potential of vomeronasal progenitor cells. OMP reactivity in the VNE of Npc1\u2212\/\u2212 mice was not affected, in contrast to that observed in the OE. Concomitantly, cathepsin-D reactivity in the VNE was virtually absent. Conclusion: Vomeronasal receptor neurons are less susceptible against NPC1 pathology than olfactory receptor neurons. Compared to control mice, however, the VNE of Npc1\u2212\/\u2212 mice displays an increased neuroregenerative potential, indicating compensatory cell renewal.<\/jats:p>","DOI":"10.3390\/ijms19113563","type":"journal-article","created":{"date-parts":[[2018,11,14]],"date-time":"2018-11-14T02:42:41Z","timestamp":1542163361000},"page":"3563","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Main Olfactory and Vomeronasal Epithelium Are Differently Affected in Niemann-Pick Disease Type C1"],"prefix":"10.3390","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4538-877X","authenticated-orcid":false,"given":"Martin","family":"Witt","sequence":"first","affiliation":[{"name":"Department of Anatomy, University of Rostock, 18057 Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ren\u00e9","family":"Thiemer","sequence":"additional","affiliation":[{"name":"Department of Anatomy, University of Rostock, 18057 Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Meyer","sequence":"additional","affiliation":[{"name":"Department of Anatomy, University of Rostock, 18057 Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Schmitt","sequence":"additional","affiliation":[{"name":"Department of Anatomy, University of Rostock, 18057 Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Wree","sequence":"additional","affiliation":[{"name":"Department of Anatomy, University of Rostock, 18057 Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2018,11,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1016\/0925-4439(91)90069-L","article-title":"Type C Niemann-Pick disease: Spectrum of phenotypic variation in disruption of intracellular LDL-derived cholesterol processing","volume":"1096","author":"Vanier","year":"1991","journal-title":"Biochim. 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