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Oligodendrocyte progenitor cells (OPCs) isolated from the cortex of TS mice showed greater L\u2010type Ca<jats:sup>++<\/jats:sup> influx and displayed characteristics suggestive of advanced maturation compared to control OPCs, including a more complex morphology and higher levels of myelin protein expression. Consistent with this, expression of Cav1.2 channels bearing the TS mutation in wild\u2010type OPCs triggered process formation and promoted oligodendrocyte\u2013neuron interaction via the activation of Ca<jats:sup>++<\/jats:sup>\/calmodulin\u2010dependent protein kinase II. To ascertain whether accelerated OPC maturation correlated with functional enhancements, we examined myelination in the TS brain at different postnatal time points. The expression of myelin proteins was significantly higher in the corpus callosum, cortex and striatum of TS animals, and immunohistochemical analysis for oligodendrocyte stage\u2010specific markers revealed an increase in the density of myelinating oligodendrocytes in several areas of the TS brain. Along the same line, electron microscopy studies in the corpus callosum of TS animals showed significant increases both in the percentage of myelinated axons and in the thickness of myelin sheaths. In summary, these data indicate that OPC development and oligodendrocyte myelination is enhanced in the brain of TS mice, and suggest that this mouse model of a syndromic ASD is a useful tool to explore the role of L\u2010type Ca<jats:sup>++<\/jats:sup> channels in myelination.<\/jats:p>","DOI":"10.1002\/glia.23468","type":"journal-article","created":{"date-parts":[[2018,8,28]],"date-time":"2018-08-28T08:34:25Z","timestamp":1535445265000},"page":"2324-2339","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Enhanced oligodendrocyte maturation and myelination in a mouse model of Timothy syndrome"],"prefix":"10.1002","volume":"66","author":[{"given":"Veronica T.","family":"Cheli","sequence":"first","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diara A.","family":"Santiago Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norma N.","family":"Zamora","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tenzing N.","family":"Lama","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vilma","family":"Spreuer","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randall L.","family":"Rasmusson","sequence":"additional","affiliation":[{"name":"Department of Physiology and Biophysics, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glenna C.","family":"Bett","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgia","family":"Panagiotakos","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Biophysics and Kavli Institute for Fundamental Neuroscience, Eli and Edythe Broad Center of Regeneration Medicine and Stem Cell Research School of Medicine, University of California at San Francisco  San Francisco California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9362-9454","authenticated-orcid":false,"given":"Pablo M.","family":"Paez","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute, Department of Pharmacology and Toxicology, Jacobs School of Medicine and Biomedical Sciences The State University of New York, University at Buffalo  Buffalo New York"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,26]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainresrev.2004.12.005"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.490360111"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1112667108"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2015.01.016"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/35012083"},{"issue":"3","key":"e_1_2_7_7_1","first-page":"135","article-title":"A mouse model of Timothy syndrome: a complex autistic disorder resulting from a point mutation in Cav1.2","volume":"5","author":"Bett C. 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