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  4. Monomeric Huntingtin Exon 1 Has Similar Overall Structural Features for Wild-Type and Pathological Polyglutamine Lengths
 
research article

Monomeric Huntingtin Exon 1 Has Similar Overall Structural Features for Wild-Type and Pathological Polyglutamine Lengths

Warner IV, John Blaine  
•
Ruff, Kiersten
•
Siong Tan, Piau
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2017
Journal of the American Chemical Society

Huntington’s disease is caused by expansion of a polyglutamine (polyQ) domain within exon 1 of the huntingtin gene (Httex1). A popular hypothesis is that the Httex1 protein undergoes sharp conformational changes as the polyQ length exceeds a threshold of 36 residues. We test this hypothesis by combining novel semi-synthesis strategies with state-of-the-art single molecule Förster resonance energy transfer measurements on biologically relevant Httex1 proteins of five different polyQ lengths. Our results, integrated with atomistic simulations, negate the hypothesis of a sharp, polyQ length-dependent change in the structure of monomeric Httex1. Instead, they support a continuous global compaction with increasing polyQ length and this derives from increased prominence of the globular polyQ domain. More specifically, we show that that monomeric Httex1 adopts tadpole-like architectures for polyQ lengths above and beyond the pathological threshold. Additionally, our results suggest that higher order homo- and / or heterotypic interactions within distinct sub-populations of neurons are likely to be the main source of sharp polyQ length-dependencies of HD. These findings pave the way for uncovering the true structural basis of Httex1-mediated neurotoxicity.

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Type
research article
DOI
10.1021/jacs.7b06659
Web of Science ID

WOS:000413503300024

Author(s)
Warner IV, John Blaine  
Ruff, Kiersten
Siong Tan, Piau
Lemke, Edward A.
Pappu, Rohit V.
Lashuel, Hilal A.  
Date Issued

2017

Published in
Journal of the American Chemical Society
Volume

139

Issue

41

Start page

14456

End page

14469

Subjects

Huntington’s disease

•

Huntingtin

•

neurodegeneration

•

neurodegenerative diseases

•

single molecule FRET

•

atomistic simulations

•

polyglutamine

•

semi synthesis

Editorial or Peer reviewed

REVIEWED

Written at

EPFL

EPFL units
LMNN  
Available on Infoscience
September 22, 2017
Use this identifier to reference this record
https://infoscience.epfl.ch/handle/20.500.14299/140797
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