title: A novel transcriptional regulator of myelin gene expression: implications for neurodevelopmental disorders.
abstract: Myelination is critical for normal functioning of mammalian central nervous system. Central nervous system myelin is created and maintained by oligodendrocytes. Protein expression patterns change as the oligodendrocyte progenitors differentiate into myelinating oligodendrocytes. Several proteins, including the cell surface proteoglycan NG2, proteolipid protein, myelin basic protein, and myelin-associated glycoprotein are critical for normal myelination. The molecular regulation of myelination is for the most part unknown, although several transcription factors have been identified as regulating myelin protein expression. We have identified a known transcriptional regulator, methyl-CpG-binding protein 2, as regulating myelin specific gene expression in a transgenic mouse. Our findings show a potential role for myelin in the pathophysiology of methyl-CpG-binding protein 2 mutation-associated disorders.
authors.affiliation: Faculty of Pharmacy, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: Parvez
authors.initials: P
authors.lastname: Vora
authors.forename: Raena
authors.initials: R
authors.lastname: Mina
authors.forename: Michael
authors.initials: M
authors.lastname: Namaka
authors.forename: Emma E
authors.initials: EE
authors.lastname: Frost
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D001923:Brain Chemistry / Q000235:genetics; D002478:Cells, Cultured; D002658:Developmental Disabilities / Q000235:genetics* / Q000378:metabolism; D005260:Female; D018507:Gene Expression Regulation, Developmental / Q000235:genetics*; D051783:Methyl-CpG-Binding Protein 2 / Q000096:biosynthesis / Q000172:deficiency / Q000235:genetics* / Q000302:isolation & purification; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D009186:Myelin Sheath / Q000235:genetics* / Q000378:metabolism; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology; D051381:Rats; D017207:Rats, Sprague-Dawley
chemical_list: C497393:Mecp2 protein, mouse; C497394:Mecp2 protein, rat; D051783:Methyl-CpG-Binding Protein 2