Article
Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat.
Brain : a journal of neurology - 1 Dec 2015
Clemens Laura E, Weber Jonasz J, Wlodkowski Tanja T, Yu-Taeger Libo, Michaud Magali, Calaminus Carsten, Eckert Schamim H, Gaca Janett, Weiss Andreas, Magg Janine C D, Jansson Erik K H, Eckert Gunter P, Pichler Bernd J, Bordet Thierry, Pruss Rebecca M, Riess Olaf, Nguyen Huu P
Abstract excerpt
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...
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