Article
Experimental mutagenesis of huntingtin to map cleavage sites: different outcomes in cell and mouse models.
Journal of Huntington's disease - 1 Jan 2014
Tebbenkamp Andrew T N, Xu Guilian, Siemienski Zoe B, Janus Christopher, Fromholt Susan E, Brown Hilda H, Swing Debbie, Tessarollo Lino, Borchelt David R
Abstract excerpt
BACKGROUND: N-terminal cleavage products of mutant huntingtin (htt) generate pathologic neuronal inclusion bodies. The precise length of the htt fragment, termed Cp-A/1, that produces HD pathologic inclusions is unknown. OBJECTIVE: We sought to elucidate the protein sequence elements within the N-terminus of htt that mediate its proteolysis based on a model in which engineered htt fragments terminating at residue...
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