Article
The dynamics of early-state transcriptional changes and aggregate formation in a Huntington's disease cell model.
BMC genomics - 12 May 2017
van Hagen Martijn, Piebes Diewertje G E, de Leeuw Wim C, Vuist Ilona M, van Roon-Mom Willeke M C, Moerland Perry D, Verschure Pernette J
Abstract excerpt
BACKGROUND: Huntington's disease (HD) is a fatal neurodegenerative disorder caused by a CAG expansion in the Huntingtin (HTT) gene. Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. To understand the...
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