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We investigated the possibility that the expression of mutant Cx32 in other cells besides myelinating Schwann cells contributes to the development of demyelination. Human Cx32 was expressed in transgenic mice using a rat<jats:italic>myelin protein zero<\/jats:italic>(<jats:italic>Mpz<\/jats:italic>) promoter, which is exclusively expressed by myelinating Schwann cells. Male mice expressing the human transgene were crossed with female<jats:italic>Gjb1\/cx32<\/jats:italic>-null mice; the resulting male offspring were all<jats:italic>cx32<\/jats:italic>-null (on the X chromosome), and one-half were transgene positive. In these transgenic mice, all of the Cx32 was derived from the expression of the transgene and was found in the sciatic nerve but not in the spinal cord or the liver. Furthermore, the Cx32 protein was properly localized (within incisures and paranodes) in myelinating Schwann cells. Finally, the expression of human Cx32 protein \u201crescued\u201d the phenotype of<jats:italic>cx32<\/jats:italic>-null mice, because the transgenic mice have significantly fewer demyelinated or remyelinated axons than their nontransgenic littermates. These results indicate that the loss of Schwann-cell-autonomous expression of Cx32 is sufficient to account for demyelination in CMT1X.<\/jats:p>","DOI":"10.1523\/jneurosci.3082-04.2005","type":"journal-article","created":{"date-parts":[[2005,2,9]],"date-time":"2005-02-09T21:52:47Z","timestamp":1107985967000},"page":"1550-1559","source":"Crossref","is-referenced-by-count":79,"title":["Transgenic Expression of Human<i>Connexin32<\/i>in Myelinating Schwann Cells Prevents Demyelination in<i>Connexin32<\/i>-Null Mice"],"prefix":"10.1523","volume":"25","author":[{"given":"Steven S.","family":"Scherer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi-Tian","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albee","family":"Messing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Willecke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth H.","family":"Fischbeck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda Jo Bone","family":"Jeng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"393","published-online":{"date-parts":[[2005,2,9]]},"reference":[{"key":"2023041303105889000_25.6.1550.1","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1097\/00005072-199907000-00004","volume":"58","year":"1999","journal-title":"J Neuropathol Exp Neurol"},{"key":"2023041303105889000_25.6.1550.2","doi-asserted-by":"crossref","first-page":"4545","DOI":"10.1523\/JNEUROSCI.17-12-04545.1997","volume":"17","year":"1997","journal-title":"J Neurosci"},{"key":"2023041303105889000_25.6.1550.3","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.142.4.1095"},{"key":"2023041303105889000_25.6.1550.4","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9568.1999.00752.x"},{"key":"2023041303105889000_25.6.1550.5","doi-asserted-by":"publisher","DOI":"10.1126\/science.8266101"},{"key":"2023041303105889000_25.6.1550.6","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/120.5.813"},{"key":"2023041303105889000_25.6.1550.7","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1212\/WNL.50.4.1074","volume":"50","year":"1998","journal-title":"Neurology"},{"key":"2023041303105889000_25.6.1550.8","unstructured":"Boyd IA, Davey MR (1968) Composition of peripheral nerves, p 57. 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