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

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2021

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Huang, Bin
Guo, Qiang
Cheng, Jingdong
Engler, Tatjana
Maurer, Melanie
Pautsch, Alexander
Kochanek, Stefan
Fernández-Busnadiego, Rubén
et al.

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Structure. Cell Press. 2021, 29(8), pp. 804-809.e5. ISSN 0969-2126. eISSN 1878-4186. Available under: doi: 10.1016/j.str.2021.04.003

Zusammenfassung

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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570 Biowissenschaften, Biologie

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ISO 690HUANG, Bin, Qiang GUO, Marie L. NIEDERMEIER, Jingdong CHENG, Tatjana ENGLER, Melanie MAURER, Alexander PAUTSCH, Florian STENGEL, Stefan KOCHANEK, Rubén FERNÁNDEZ-BUSNADIEGO, 2021. Pathological polyQ expansion does not alter the conformation of the Huntingtin-HAP40 complex. In: Structure. Cell Press. 2021, 29(8), pp. 804-809.e5. ISSN 0969-2126. eISSN 1878-4186. Available under: doi: 10.1016/j.str.2021.04.003
BibTex
@article{Huang2021-08-05Patho-54848,
  year={2021},
  doi={10.1016/j.str.2021.04.003},
  title={Pathological polyQ expansion does not alter the conformation of the Huntingtin-HAP40 complex},
  number={8},
  volume={29},
  issn={0969-2126},
  journal={Structure},
  pages={804--809.e5},
  author={Huang, Bin and Guo, Qiang and Niedermeier, Marie L. and Cheng, Jingdong and Engler, Tatjana and Maurer, Melanie and Pautsch, Alexander and Stengel, Florian and Kochanek, Stefan and Fernández-Busnadiego, Rubén}
}
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