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However, the <jats:italic>Dst<\/jats:italic>\u2010expressing cell type(s) for <jats:italic>dt<\/jats:italic> phenotypes have not been well characterized. To address the questions whether the disruption of <jats:italic>Dst<\/jats:italic> in Schwann cells induces movement disorders and how much impact does it have on <jats:italic>dt<\/jats:italic> phenotypes, we generated <jats:italic>Dst<\/jats:italic> conditional knockout (cKO) mice using <jats:italic>P0\u2010Cre<\/jats:italic> transgenic mice and <jats:italic>Dst<\/jats:italic> gene trap mice. First, we assessed the <jats:italic>P0\u2010Cre<\/jats:italic> transgene\u2010dependent Cre recombination using <jats:italic>tdTomato<\/jats:italic> reporter mice and then confirmed the preferential tdTomato expression in Schwann cells. In the <jats:italic>Dst<\/jats:italic> cKO mice, <jats:italic>Dst<\/jats:italic> mRNA expression was significantly decreased in Schwann cells, but it was intact in most of the sensory neurons in the dorsal root ganglion. Next, we analyzed the phenotype of <jats:italic>Dst<\/jats:italic> cKO mice. They exhibited a normal motor phenotype during juvenile periods, and thereafter, started exhibiting an ataxia. Behavioral tests and electrophysiological analyses demonstrated impaired motor abilities and slowed motor nerve conduction velocity in <jats:italic>Dst<\/jats:italic> cKO mice, but these mice did not manifest dystonic movements. Electron microscopic observation of the PNS of <jats:italic>Dst<\/jats:italic> cKO mice revealed significant numbers of hypomyelinated axons and numerous infiltrating macrophages engulfing myelin debris. These results indicate that <jats:italic>Dst<\/jats:italic> is important for normal PNS myelin organization and <jats:italic>Dst<\/jats:italic> disruption in Schwann cells induces late\u2010onset neuropathy and sensory ataxia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Main points<\/jats:title><jats:p><jats:list list-type=\"bullet\">\n<jats:list-item><jats:p><jats:italic>Dystonin<\/jats:italic> (<jats:italic>Dst<\/jats:italic>) disruption in Schwann cells results in late\u2010onset neuropathy and sensory ataxia.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p><jats:italic>Dst<\/jats:italic> in Schwann cells is important for normal myelin organization in the peripheral nervous system.<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p><\/jats:sec>","DOI":"10.1002\/glia.23843","type":"journal-article","created":{"date-parts":[[2020,5,23]],"date-time":"2020-05-23T15:15:55Z","timestamp":1590246955000},"page":"2330-2344","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Disruption of <scp><i>dystonin<\/i><\/scp> in Schwann cells results in late\u2010onset neuropathy and sensory ataxia"],"prefix":"10.1002","volume":"68","author":[{"given":"Masao","family":"Horie","sequence":"first","affiliation":[{"name":"Division of Neurobiology and Anatomy Graduate School of Medical and Dental Sciences, Niigata University  Niigata Japan"},{"name":"Department of Morphological Sciences Kagoshima University  Kagoshima Japan"},{"name":"Department of Nursing Niigata College of Nursing  Niigata 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