論文

2017年12月

Enhanced expression of Pafah1b1 causes over-migration of cerebral cortical neurons into the marginal zone

BRAIN STRUCTURE & FUNCTION
  • Kei-ichi Katayama
  • ,
  • Kanehiro Hayashi
  • ,
  • Seika Inoue
  • ,
  • Kazushige Sakaguchi
  • ,
  • Kazunori Nakajima

222
9
開始ページ
4283
終了ページ
4291
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1007/s00429-017-1497-9
出版者・発行元
SPRINGER HEIDELBERG

Mutations of PAFAH1B1 cause classical lissencephaly in humans. In addition, duplications and triplications of PAFAH1B1 are found in individuals with intellectual disability and other neurological disorders suggesting that proper brain development is highly sensitive to the PAFAH1B1 dosage. To examine the effect of PAFAH1B1 over-dosage in neural development, especially in migration of neurons and layer formation during cerebral cortical development, we overexpressed Pafah1b1 in migrating neurons in the mouse embryonic cortex using in utero electroporation. Enhanced expression of Pafah1b1 in radially-migrating neurons resulted in their over-migration into the marginal zone. Neurons that invaded the marginal zone were oriented abnormally. Layer distribution of Pafaha1b1-overexpressing neurons shifted more superficially than control neurons. Some of the Pafaha1b1-overexpressing future layer 4 neurons changed their positions to layers 2/3. Furthermore, they also changed their layer marker expression from layer 4 to layers 2/3. These results suggest that overexpression of Pafah1b1 affects the migration of neurons and disrupts layer formation in the developing cerebral cortex, and further support the idea that appropriate dosage of Pafah1b1 is crucial for the proper development of the brain.

Web of Science ® 被引用回数 : 3

リンク情報
DOI
https://doi.org/10.1007/s00429-017-1497-9
Web of Science
https://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=JSTA_CEL&SrcApp=J_Gate_JST&DestLinkType=FullRecord&KeyUT=WOS:000415357100027&DestApp=WOS_CPL

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