Structure and Dynamics of Huntingtin. A Segmental Labelling Approach
Huntington's disease (HD) is a neurodegenerative disorder caused when the number of consecutive glutamines in the poly-glutamine (poly-Q) tract of the huntingtin exon1 (httex1) exceeds 35. In addition of the poly-Q, httex1 contains two poly-Proline (poly-P) tracts. Due to its flexibility and the low complexity of its sequence, the structural characterization of httex1 is challenging, and the structural bases of its pathological threshold remain poorly understood. In this project we will overcome these problems by applying contrast variation SANS experiments to multiple segmentally labelled versions of the protein. We will exploit the control of the amino acid composition offered by the Cell-Free protein synthesis in order to selectively deuterate httex1 repeats. By deuterating the repeats individually we will obtain information about their structural properties. When simultaneously deuterating both repeats we will probe the relative localization of the homopolymeric regions. The ensemble of SANS profiles will be combined with SAXS and NMR data in order to derive ensemble models of httex1 reporting on the overall shape of the protein and insights into the structural bases of HD.
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BERNADO Pau; GABEL Frank; Xamuel L. Lund; MARTEL Anne and MATSARSKAIA Olga. (2021). Structure and Dynamics of Huntingtin. A Segmental Labelling Approach. Institut Laue-Langevin (ILL) doi:10.5291/ILL-DATA.8-03-1020
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