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  Pathological polyQ expansion does not alter the conformation of the Huntingtin-HAP40 complex

Huang, B., Guo, Q., Niedermeier, M. L., Cheng, J., Engler, T., Maurer, M., et al. (2021). Pathological polyQ expansion does not alter the conformation of the Huntingtin-HAP40 complex. Structure, 29(8), 804-809. doi:10.1016/j.str.2021.04.003.

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 Creators:
Huang, Bin1, Author
Guo, Qiang2, Author
Niedermeier, Marie L.1, Author
Cheng, Jingdong1, Author
Engler, Tatjana1, Author
Maurer, Melanie1, Author
Pautsch, Alexander1, Author
Baumeister, Wolfgang2, Author              
Stengel, Florian1, Author
Kochanek, Stefan1, Author
Fernandez-Busnadiego, Ruben2, Author              
Affiliations:
1external, ou_persistent22              
2Baumeister, Wolfgang / Molecular Structural Biology, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565142              

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Free keywords: PROTEIN STABILITYBiochemistry & Molecular Biology; Biophysics; Cell Biology;
 Abstract: The abnormal amplification of a CAG repeat in the gene coding for huntingtin (HTT) leads to Huntington's disease (HD). At the protein level, this translates into the expansion of a polyglutamine (polyQ) stretch located at the HTT N terminus, which renders HTT aggregation prone by unknown mechanisms. Here we investigated the effects of polyQ expansion on HTT in a complex with its stabilizing interaction partner huntingtin-associated protein 40 (HAP40). Surprisingly, our comprehensive biophysical, crosslinking mass spectrometry and cryo-EM experiments revealed no major differences in the conformation of HTT-HAP40 complexes of various polyQ length, including 17QHTT-HAP40 (wild type), 46QHTT-HAP40 (typical polyQ length in HD patients), and 128QHTT-HAP40 (extreme polyQ length). Thus, HTT polyQ expansion does not alter the global conformation of HTT when associated with HAP40.

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Language(s): eng - English
 Dates: 2021
 Publication Status: Published in print
 Pages: 11
 Publishing info: -
 Table of Contents: -
 Rev. Type: -
 Identifiers: ISI: 000682520300005
DOI: 10.1016/j.str.2021.04.003
 Degree: -

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Title: Structure
  Other : Structure
Source Genre: Journal
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Publ. Info: London : Cell Press
Pages: - Volume / Issue: 29 (8) Sequence Number: - Start / End Page: 804 - 809 Identifier: ISSN: 0969-2126
CoNE: https://pure.mpg.de/cone/journals/resource/954927002244_1