{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:43:29Z","timestamp":1790775809708,"version":"4.1.0"},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2002,5,24]],"date-time":"2002-05-24T00:00:00Z","timestamp":1022198400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2002,7,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Dystonia musculorum (<jats:italic>dt<\/jats:italic>) is an autosomal recessive sensory neuropathy in mice resulting from a mutation in the gene encoding the cytoskeletal linker protein Bpag1. In addition to neurodegeneration, <jats:italic>dt<\/jats:italic> mice display myelination abnormalities in the peripheral nervous system. In this report we investigated whether myelination abnormalities are also present in the central nervous system of <jats:italic>dt<\/jats:italic><jats:sup><jats:italic>Tg4<\/jats:italic><\/jats:sup> mice. Transcripts for both neural isoforms of Bpag1 (a1 and a2) were detected in optic nerves and spinal cords of wild\u2010type mice. Light microscopy of resin\u2010embedded thin sections revealed a reduction in myelinated axons in both optic nerves and spinal cords in <jats:italic>dt<\/jats:italic><jats:sup><jats:italic>Tg4<\/jats:italic><\/jats:sup> mice. As well, hypermyelinated axons were detected in these tissues. Ultrastructural analysis of optic nerves and spinal cords from <jats:italic>dt<\/jats:italic><jats:sup><jats:italic>Tg4<\/jats:italic><\/jats:sup> mice revealed an increase in the number of amyelinated axons, the presence of hypo\u2010 and hypermyelinated axons, and redundant myelin that course away from axons. Changes in the level of myelin proteins accompanied the morphological alterations. Myelin\u2010associated glycoprotein levels were reduced in optic nerves of <jats:italic>dt<\/jats:italic><jats:sup><jats:italic>Tg4<\/jats:italic><\/jats:sup> mice, and myelin basic protein levels were altered in optic nerves, sciatic nerves, and spinal cords of affected mice. Short\u2010term cultures of oligodendrocytes derived from <jats:italic>dt<\/jats:italic><jats:sup><jats:italic>Tg4<\/jats:italic><\/jats:sup> mice did not show morphological alterations. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.10289","type":"journal-article","created":{"date-parts":[[2002,8,25]],"date-time":"2002-08-25T23:33:49Z","timestamp":1030318429000},"page":"233-242","source":"Crossref","is-referenced-by-count":16,"title":["Alterations in myelination in the central nervous system of dystonia musculorum mice"],"prefix":"10.1002","volume":"69","author":[{"given":"Ron","family":"Saulnier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"De Repentigny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Voon 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