Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease

Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease

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 olig...

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Bibliographic Details
Main Authors: Kenneth W. Drombosky, Sascha Rode, Ravi Kodali, Tija C. Jacob, Michael J. Palladino, Ronald Wetzel
Format: Article
Language:English
Published: Elsevier 2018-12-01
Series:Neurobiology of Disease
Subjects:
Polyglutamine
β-Hairpin
Oligomers
Primary neurons
Drosophila
Thioflavin T
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996118305084

Internet

http://www.sciencedirect.com/science/article/pii/S0969996118305084

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