SMAD transcription factors are altered in cell models of HD and regulate HTT expression

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• 作者：KR Bowles^a ; ^{kathryn.bowles@mssm.edu} ; T Stone^a ; ^{stonetc@cardiff.ac.uk} ; P Holmans^a ; ^{holmanspa@cardiff.ac.uk} ; ND Allen^b ; ^{allennd@cardiff.ac.uk} ; SB Dunnett^c ; ^{dunnettsb@Cardiff.ac.uk} ; L Jones^a ; ^{jonesl1@cardiff.ac.uk}
• 关键词：Huntington's Disease ; Transcription ; EGF ; TGF-beta ; SMAD ; Kinase signalling
• 刊名：Cellular Signalling
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：31
• 期：Complete
• 页码：1-14
• 全文大小：2783 K
• 卷排序：31

文摘

Dysregulation of TGFβ signalling is identified in mouse and human cell models of Huntington's disease. Characterisation of this dysregulation in downstream SMAD transcription factors is presented. SMAD3 binds to the HTT promotor and can regulate HTT expression in the absence of a CAG expansion.