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We established 90\u2010day dorsal root ganglia (DRG) cultures from a rat model of Charcot\u2013Marie\u2013Tooth type\u20031A (CMT1A) neuropathy to evaluate the structure of myelin and axons, and the expression of myelin\u2010related proteins and cytoskeletal components, by morphological and molecular techniques. Both wild\u2010type and CMT1A cultures were rich in myelinated fibres. Affected cultures showed dysmyelinated internodes and focal myelin swellings. Furthermore, uncompacted myelin and smaller axons with increased neurofilament (NF) density were found by electron microscopy, and Western blots showed higher levels of nonphosphorylated NF. Confocal microscopy demonstrated an abnormal distribution of the myelin\u2010associated glycoprotein which, instead of being expressed at the noncompact myelin level, showed focal accumulation along the internodes while other myelin proteins were normally distributed. These findings suggest that CMT1A DRG cultures, similarly to the animal model and human disease, undergo axonal atrophy over a period of time. This model may be utilized to study the molecular changes underlying demyelination and secondary axonal impairment. As axonal damage may occur after just 3\u2003months and tissue cultures represent a strictly controlled environment, this model may be ideal for testing neuroprotective therapies.<\/jats:p>","DOI":"10.1111\/j.1460-9568.2006.04666.x","type":"journal-article","created":{"date-parts":[[2006,3,16]],"date-time":"2006-03-16T15:26:15Z","timestamp":1142522775000},"page":"1445-1452","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Axonal damage and demyelination in long\u2010term dorsal root ganglia cultures from a rat model of Charcot\u2013Marie\u2013Tooth type\u20031A disease"],"prefix":"10.1111","volume":"23","author":[{"given":"Lucilla","family":"Nobbio","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianfranco","family":"Gherardi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiziana","family":"Vigo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario","family":"Passalacqua","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edon","family":"Melloni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Abbruzzese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianluigi","family":"Mancardi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus\u2010Armin","family":"Nave","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angelo","family":"Schenone","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,3,16]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.147.6.1123"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1007010205037"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.32.2.439"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1529-8027.2001.01013.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4547(19990415)56:2<213::AID-JNR11>3.0.CO;2-U"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.298.5593.556"},{"key":"e_1_2_7_8_1","first-page":"1094","volume-title":"Peripheral Neuropathy","author":"Dyck P.J.","year":"1993"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.39.10.1302"},{"key":"e_1_2_7_10_1","first-page":"34","article-title":"The nature of myelinated nerve fiber degeneration in dominantly inherited hypertrophic neuropathy","volume":"49","author":"Dyck P.J.","year":"1974","journal-title":"Mayo Clin. 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