論文

査読有り 国際誌
2019年7月23日

Loss of Wwox Causes Defective Development of Cerebral Cortex with Hypomyelination in a Rat Model of Lethal Dwarfism with Epilepsy.

International journal of molecular sciences
  • Yuki Tochigi
  • ,
  • Yutaka Takamatsu
  • ,
  • Jun Nakane
  • ,
  • Rika Nakai
  • ,
  • Kentaro Katayama
  • ,
  • Hiroetsu Suzuki

20
14
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.3390/ijms20143596

WW domain-containing oxidoreductase (Wwox) is a putative tumor suppressor. Several germline mutations of Wwox have been associated with infant neurological disorders characterized by epilepsy, growth retardation, and early death. Less is known, however, about the pathological link between Wwox mutations and these disorders or the physiological role of Wwox in brain development. In this study, we examined age-related expression and histological localization of Wwox in forebrains as well as the effects of loss of function mutations in the Wwox gene in the immature cortex of a rat model of lethal dwarfism with epilepsy (lde/lde). Immunostaining revealed that Wwox is expressed in neurons, astrocytes, and oligodendrocytes. lde/lde cortices were characterized by a reduction in neurite growth without a reduced number of neurons, severe reduction in myelination with a reduced number of mature oligodendrocytes, and a reduction in cell populations of astrocytes and microglia. These results indicate that Wwox is essential for normal development of neurons and glial cells in the cerebral cortex.

リンク情報
DOI
https://doi.org/10.3390/ijms20143596
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/31340538
PubMed Central
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678113

エクスポート
BibTeX RIS