Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat.

  title={Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat.},
  author={Laura Emily Clemens and Jonasz Jeremiasz Weber and Tanja T Wlodkowski and Libo Yu-Taeger and Magali Michaud and Carsten Calaminus and Schamim H. Eckert and Janett Gaca and Andreas M. Weiss and Janine C. D. Magg and Erik Karl H{\aa}kan Jansson and Gunter P Eckert and Bernd J. Pichler and Thierry Bordet and Rebecca M. Pruss and Olaf Riess and Huu Phuc Nguyen},
  journal={Brain : a journal of neurology},
  volume={138 Pt 12},
Huntington's disease is a fatal human neurodegenerative disorder caused by a CAG repeat expansion in the HTT gene, which translates into a mutant huntingtin protein. A key event in the molecular pathogenesis of Huntington's disease is the proteolytic cleavage of mutant huntingtin, leading to the accumulation of toxic protein fragments. Mutant huntingtin cleavage has been linked to the overactivation of proteases due to mitochondrial dysfunction and calcium derangements. Here, we investigated… CONTINUE READING
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2014 emerging science abstracts

  • E Dessaud, A Carole, S Bruno, B Patrick, P Rebecca, V Cuvier
  • Neurology 2014;
  • 2014
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