[HTML][HTML] Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease
C Landles, K Sathasivam, A Weiss, B Woodman… - Journal of Biological …, 2010 - ASBMB
Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington
disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal
fragment has not been determined. Using a panel of anti-huntingtin antibodies, we
employed an unbiased approach to generate proteolytic cleavage maps of mutant and wild-
type huntingtin in the HdhQ150 knock-in mouse model of HD. We identified 14 prominent N-
terminal fragments, which, in addition to the full-length protein, can be readily detected in …
disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal
fragment has not been determined. Using a panel of anti-huntingtin antibodies, we
employed an unbiased approach to generate proteolytic cleavage maps of mutant and wild-
type huntingtin in the HdhQ150 knock-in mouse model of HD. We identified 14 prominent N-
terminal fragments, which, in addition to the full-length protein, can be readily detected in …