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DM\u201020 is also present in myelin\u2010forming and non\u2010myelin\u2010forming Schwann cells, olfactory nerve ensheathing cells, some glial cell lines and cardiac myocytes. Molecular studies suggest the existence of a PLP gene family with sequence similarities between molecules of different species. Such studies also lend credence to the suggestion that PLP and\/or DM\u201020 may function as a membrane pore. Mutations in the PLP gene occur in several animal species and cause severe pleiotropic effects on myelination. In man this presents as Pelizaeus\u2010Merzbacher disease (PMD). The phenotype of such mutants is characterized by dysmyelination with myelin of abnormal periodicity, paucity of mature oligodendrocytes and astrocytosis. Duplication of the PLP gene in transgenic animals or in one form of PMD also results in dysmyelination. X\u2010linked spastic paraplegia (SPG2) is allelic to PMD and is associated with PLP mutations in which the levels of the DM\u201020 isoform are probably relatively normal. The effects of PLP gene dosage on CNS myelination can be compared in many ways to the variety of phenotypes in the PNS in hereditary neuropathies of the Charcot\u2010Marie\u2010Tooth type in which the peripheral myelin\u201022 gene is mutated.<\/jats:p>","DOI":"10.1111\/j.1365-2990.1995.tb01034.x","type":"journal-article","created":{"date-parts":[[2008,5,12]],"date-time":"2008-05-12T09:44:54Z","timestamp":1210585494000},"page":"85-96","source":"Crossref","is-referenced-by-count":52,"title":["The proteolipid protein gene"],"prefix":"10.1111","volume":"21","author":[{"given":"I. 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