APC conditional knock-out mouse is a model of infantile spasms with elevated neuronal β-catenin levels, neonatal spasms, and chronic seizures

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• 作者：Antonella Pirone^a ; Jonathan Alexander^a ; ^b ; Lauren A. Lau^a ; ^b ; David Hampton^a ; Andrew Zayachkivsky^c ; Amy Yee^d ; Audrey Yee^e ; Michele H. Jacob^a ; ^{Michele.Jacob@Tufts.edu} ; Chris G. Dulla^a ; ^{Chris.Dulla@Tufts.edu}
• 关键词：Infantile spasms ; Epilepsy ; β-Catenin ; Wnt signaling ; Cortex
• 刊名：Neurobiology of Disease
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：98
• 期：Complete
• 页码：149-157
• 全文大小：1336 K
• 卷排序：98

文摘

We generated a novel mouse model (APC cKO) that recapitulates features of human IS. APC deletion increases β-catenin levels and frequency of EPSCs. Neonatal spasms and aberrant cortical activity progress to spontaneous seizures. We identify novel molecular pathways (APC/β-catenin/Wnt signaling) in a model of IS.