Downregulation of CNPase in a MeCP2 deficient mouse model of Rett syndrome

Neurol Res. 2012 Mar;34(2):107-13. doi: 10.1179/016164111X13214359296301. Epub 2012 Jan 13.

Abstract

Objectives: 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.

Conclusion: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 2',3'-Cyclic-Nucleotide Phosphodiesterases / biosynthesis*
  • Animals
  • Blotting, Western
  • Brain / metabolism*
  • Connexins / biosynthesis
  • Disease Models, Animal
  • Down-Regulation
  • Glial Fibrillary Acidic Protein / biosynthesis
  • Methyl-CpG-Binding Protein 2 / deficiency
  • Methyl-CpG-Binding Protein 2 / genetics*
  • Mice
  • Mice, Mutant Strains
  • Mutation
  • RNA, Messenger / analysis
  • Real-Time Polymerase Chain Reaction
  • Rett Syndrome / genetics
  • Rett Syndrome / metabolism*

Substances

  • Connexins
  • Glial Fibrillary Acidic Protein
  • Mecp2 protein, mouse
  • Methyl-CpG-Binding Protein 2
  • RNA, Messenger
  • 2',3'-Cyclic-Nucleotide Phosphodiesterases