Article
Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease.
Neurobiology of disease - 1 Dec 2018
Drombosky Kenneth W, Rode Sascha, Kodali Ravi, Jacob Tija C, Palladino Michael J, Wetzel Ronald
Abstract excerpt
In Huntington disease (HD), an expanded polyglutamine (polyQ > 37) sequence within huntingtin (htt) exon1 leads to enhanced disease risk. It has proved difficult, however, to determine whether the toxic form generated by polyQ expansion is a misfolded or avid-binding monomer, an α-helix-rich oligomer, or a β-sheet-rich amyloid fibril. Here we describe an engineered htt exon1 analog featuring a short polyQ...
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