title: Downregulation of CNPase in a MeCP2 deficient mouse model of Rett syndrome.
abstract: OBJECTIVE
  To investigate the possible target genes of methyl-CpG-binding protein 2 (MeCP2) that contribute to Rett syndrome (RTT).
  
  
  METHODS
  Brain tissues were taken from Mecp2(308/Y) mice or control mice and then subjected to real-time  quantitative reverse transcriptase polymerase chain reaction (RT-PCR), immunohistochemical staining, and Western blot analysis for connexin (Cx)43, Cx45, Cx40, Cx32, 2,3-cyclic nucleotide 3-phosphohydrolase (CNPase), and glial fibrillary acidic protein (GFAP).
  
  
  RESULTS
  The expression  of CNPase in subcortical white matter and hippocampi was lower in RTT mice compared to control mice at both mRNA and protein levels. In contrast, the expression of Cx43, Cx40, Cx45, Cx32, or GFAP was not altered in RTT mice compared to control mice.
  
  
  CONCLUSIONS
  The downregulation  of CNPase expression in the brain may be a possible consequence of Mecp2 gene mutation, and the indicated dysfunction of the oligodendrocytes in the subcortical white matter and hippocampi may be involved in RTT pathogenesis.
authors.affiliation: Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou, Zhejiang 310003, China. wuwei120@sina.com
authors.forename: Wei
authors.initials: W
authors.lastname: Wu
authors.forename: Weizhong
authors.initials: W
authors.lastname: Gu
authors.forename: Xuefeng
authors.initials: X
authors.lastname: Xu
authors.forename: Shiqiang
authors.initials: S
authors.lastname: Shang
authors.forename: Zhengyan
authors.initials: Z
authors.lastname: Zhao
mesh_terms: D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases / Q000096:biosynthesis*; D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism*; D017630:Connexins / Q000096:biosynthesis; D004195:Disease Models, Animal; D015536:Down-Regulation; D005904:Glial Fibrillary Acidic Protein / Q000096:biosynthesis; D051783:Methyl-CpG-Binding Protein 2 / Q000172:deficiency / Q000235:genetics*; D051379:Mice; D008817:Mice, Mutant Strains; D009154:Mutation; D012333:RNA, Messenger / Q000032:analysis; D060888:Real-Time Polymerase Chain Reaction; D015518:Rett Syndrome / Q000235:genetics / Q000378:metabolism*
chemical_list: D017630:Connexins; D005904:Glial Fibrillary Acidic Protein; C497393:Mecp2 protein, mouse; D051783:Methyl-CpG-Binding Protein 2; D012333:RNA, Messenger; D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases