{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T16:29:14Z","timestamp":1785601754129,"version":"3.56.0"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,7,1]],"date-time":"2014-07-01T00:00:00Z","timestamp":1404172800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2014,8]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Spinocerebellar ataxia type 3 (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3) is an inherited spinocerebellar ataxia caused by the expansion of trinucleotide <jats:styled-content style=\"fixed-case\">CAG<\/jats:styled-content> repeats in the gene encoding ataxin\u20103. The clinical manifestations of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3 include peripheral neuropathy, which is an important cause of disability in a subset of patients. Although the loss of neurones in the dorsal root ganglion (<jats:styled-content style=\"fixed-case\">DRG<\/jats:styled-content>) has been postulated to be the cause of this neuropathy, the precise mechanism remains to be elucidated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>To clarify the clinicopathological characteristics of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3\u2010associated peripheral neuropathy, we performed nerve conduction studies and histopathological analyses. Nerve conduction studies were carried out in 18 <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3 patients. Immunohistochemical analyses of the anterior and posterior roots of the spinal cord and peripheral nerves were performed in five <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3 patients. We also employed immunohistochemistry and immunoelectron microscopy analyses with an anti\u2010polyglutamine antibody.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The mean sensory nerve action potentials of the <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3 patients were half of the normal values. The motor conduction velocities were decreased, and the distal latencies were also significantly prolonged in the nerves studied relative to the those in normal controls. Histopathological analyses detected axonal sprouting and myelin thinning in all cases. Ataxin\u20103 aggregates were found in the cytoplasm of <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>chwann cells in all of the <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3 patients examined but not in control subjects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>n addition to the previously reported neuronopathy, the results of the present study indicate that <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>chwann cells are involved in the formation of the pathogenic intracytoplasmic ataxin\u20103 protein aggregates in patients with <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>3\u2010associated neuropathy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12055","type":"journal-article","created":{"date-parts":[[2013,4,26]],"date-time":"2013-04-26T10:54:39Z","timestamp":1366973679000},"page":"628-639","source":"Crossref","is-referenced-by-count":20,"title":["<scp>S<\/scp>chwann cell involvement in the peripheral neuropathy of spinocerebellar ataxia type 3"],"prefix":"10.1111","volume":"40","author":[{"given":"Noriaki","family":"Suga","sequence":"first","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahisa","family":"Katsuno","sequence":"additional","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruki","family":"Koike","sequence":"additional","affiliation":[{"name":"Department of Neurology Nagoya University Graduate School of 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