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Article

Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease

21 Jul 2010

Abstract excerpt

Caused by a polyglutamine expansion in the huntingtin protein, Huntington's disease leads to striatal degeneration via the transcriptional dysregulation of a number of genes, including those involved in mitochondrial biogenesis. Here we show that transglutaminase 2, which is upregulated in HD, exacerbates transcriptional dysregulation by acting as a selective corepressor of nuclear genes; transglutaminase 2...

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PMID: 20665636
DOI: 10.1002/emmm.201000084

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