Article
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Nature chemical biology - 1 Jan 2010
Hutt Darren M, Herman David, Rodrigues Ana P C, Noel Sabrina, Pilewski Joseph M, Matteson Jeanne, Hoch Ben, Kellner Wendy, Kelly Jeffery W, Schmidt Andre, Thomas Philip J, Matsumura Yoshihiro, Skach William R, Gentzsch Martina, Riordan John R, Sorscher Eric J, Okiyoneda Tsukasa, Yates John R, Lukacs Gergely L, Frizzell Raymond A, Manning Gerard, Gottesfeld Joel M, Balch William E
Abstract excerpt
Chemical modulation of histone deacetylase (HDAC) activity by HDAC inhibitors (HDACi) is an increasingly important approach for modifying the etiology of human disease. Loss-of-function diseases arise as a consequence of protein misfolding and degradation, which lead to system failures. The DeltaF508 mutation in cystic fibrosis transmembrane conductance regulator (CFTR) results in the absence of the cell surface...
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