Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function
| LEADER | caa a22 4500 | ||
|---|---|---|---|
| 001 | 528785478 | ||
| 005 | 20190908091347.0 | ||
| 007 | cr unu---uuuuu | ||
| 008 | 180924s2016 xx s 000 0 eng | ||
| 024 | 7 | 0 | |a 10.3929/ethz-b-000114979 |2 doi |
| 024 | 7 | 0 | |a 10.7554/eLife.11184 |2 doi |
| 035 | |a (ETHRESEARCH)oai:www.research-collecti.ethz.ch:20.500.11850/114979 | ||
| 245 | 0 | 0 | |a Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function |h [Elektronische Daten] |c [Ravi Vijayvargia, Raquel F. Epand, Alexander Leitner, Tae-Yang Jung, Baehyun Shin, Roy Jung, Alejandro Lloret, Randy S. Atwal, Hyeongseok Lee, Jong-Min Lee, Ruedi Aebersold, Hans Hebert, Ji-Joon Song, Ihn Sik Seong] |
| 506 | |a Open access |2 ethresearch | ||
| 520 | 3 | |a The polyglutamine expansion in huntingtin protein causes Huntington's disease. Here, we investigated structural and biochemical properties of huntingtin and the effect of the polyglutamine expansion using various biophysical experiments including circular dichroism, single-particle electron microscopy and cross-linking mass spectrometry. Huntingtin is likely composed of five distinct domains and adopts a spherical α-helical solenoid where the amino-terminal and carboxyl-terminal regions fold to contain a circumscribed central cavity. Interestingly, we showed that the polyglutamine expansion increases α-helical properties of huntingtin and affects the intramolecular interactions among the domains. Our work delineates the structural characteristics of full-length huntingtin, which are affected by the polyglutamine expansion, and provides an elegant solution to the apparent conundrum of how the extreme amino-terminal polyglutamine tract confers a novel property on huntingtin, causing the disease. | |
| 540 | |a Creative Commons Attribution 4.0 International |u http://creativecommons.org/licenses/by/4.0 |2 ethresearch | ||
| 700 | 1 | |a Vijayvargia |D Ravi |e joint author | |
| 700 | 1 | |a Epand |D Raquel F. |e joint author | |
| 700 | 1 | |a Leitner |D Alexander |e joint author | |
| 700 | 1 | |a Jung |D Tae-Yang |e joint author | |
| 700 | 1 | |a Shin |D Baehyun |e joint author | |
| 700 | 1 | |a Jung |D Roy |e joint author | |
| 700 | 1 | |a Lloret |D Alejandro |e joint author | |
| 700 | 1 | |a Atwal |D Randy S. |e joint author | |
| 700 | 1 | |a Lee |D Hyeongseok |e joint author | |
| 700 | 1 | |a Lee |D Jong-Min |e joint author | |
| 700 | 1 | |a Aebersold |D Ruedi |e joint author | |
| 700 | 1 | |a Hebert |D Hans |e joint author | |
| 700 | 1 | |a Song |D Ji-Joon |e joint author | |
| 700 | 1 | |a Seong |D Ihn Sik |e joint author | |
| 773 | 0 | |t eLife |d Cambridge : eLife Sciences Publications |g 5, p. e11184 |x 2050-084X | |
| 856 | 4 | 0 | |u http://hdl.handle.net/20.500.11850/114979 |q text/html |z WWW-Backlink auf das Repository (Open access) |
| 908 | |D 1 |a Journal Article |2 ethresearch | ||
| 950 | |B ETHRESEARCH |P 856 |E 40 |u http://hdl.handle.net/20.500.11850/114979 |q text/html |z WWW-Backlink auf das Repository (Open access) | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Vijayvargia |D Ravi |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Epand |D Raquel F. |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Leitner |D Alexander |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Jung |D Tae-Yang |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Shin |D Baehyun |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Jung |D Roy |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Lloret |D Alejandro |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Atwal |D Randy S. |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Lee |D Hyeongseok |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Lee |D Jong-Min |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Aebersold |D Ruedi |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Hebert |D Hans |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Song |D Ji-Joon |e joint author | ||
| 950 | |B ETHRESEARCH |P 700 |E 1- |a Seong |D Ihn Sik |e joint author | ||
| 950 | |B ETHRESEARCH |P 773 |E 0- |t eLife |d Cambridge : eLife Sciences Publications |g 5, p. e11184 |x 2050-084X | ||
| 898 | |a BK010053 |b XK010053 |c XK010000 | ||
| 949 | |B ETHRESEARCH |F ETHRESEARCH |b ETHRESEARCH |j Journal Article |c Open access | ||