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Author, Editor(s)

Author(s):

Boeddrich, Annett
Gaumer, Sébastien
Haacke, Annette
Tzvetkov, Nikolay
Albrecht, Mario
Evert, Bernd O.
Müller, Eva C.
Lurz, Rudi
Breuer, Peter
Schugardt, Nancy
Plaßmann, Stephanie
Xu, Kexiang
Warrick, John M.
Suopanki, Jaana
Wüllner, Ullrich
Frank, Ronald
Hartl, Ulrich F.
Bonini, Nancy M.
Wanker, Erich E.

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Not MPG Author(s):

Boeddrich, Annett
Gaumer, Sébastien
Haacke, Annette
Tzvetkov, Nikolay
Evert, Bernd O.
Müller, Eva C.
Lurz, Rudi
Breuer, Peter
Schugardt, Nancy
Plaßmann, Stephanie
Xu, Kexiang
Warrick, John M.
Suopanki, Jaana
Wüllner, Ullrich
Frank, Ronald
Hartl, Ulrich F.
Bonini, Nancy M.
Wanker, Erich E.

BibTeX cite key*:

Albrecht2006e

Title

Title*:

An arginine/lysine-rich motif is crucial for VCP/p97-mediated modulation of ataxin-3 fibrillogenesis

Journal

Journal Title*:

EMBO Journal

Journal's URL:


Download URL
for the article:

http://www.nature.com/emboj/journal/v25/n7/pdf/7601043a.pdf

Language:

English

Publisher

Publisher's
Name:


Publisher's URL:


Publisher's
Address:


ISSN:


Vol, No, pp, Date

Volume*:

25

Number:

7

Publishing Date:

2006

Pages*:

1547-1558

Number of
VG Pages:


Page Start:


Page End:


Sequence Number:


DOI:


Note, Abstract, ©

Note:


(LaTeX) Abstract:

Arginine/lysine-rich motifs typically function as targeting signals for the translocation of proteins to the nucleus. Here, we demonstrate that such a motif consisting of four basic amino acids in the polyglutamine protein ataxin-3 (Atx-3) serves as a recognition site for the interaction with the molecular chaperone VCP. Through this interaction, VCP modulates the fibrillogenesis of pathogenic forms of Atx-3 in a concentration-dependent manner, with low concentrations of VCP stimulating fibrillogenesis and excess concentrations suppressing it. No such effect was observed with a mutant Atx-3 variant, which does not contain a functional VCP interaction motif. Strikingly, a stretch of four basic amino acids in the ubiquitin chain assembly factor E4B was also discovered to be critical for VCP binding, indicating that arginine/lysine-rich motifs might be generally utilized by VCP for the targeting of proteins. In vivo studies with Drosophila models confirmed that VCP selectively modulates aggregation and neurotoxicity induced by pathogenic Atx-3. Together, these results define the VCP–Atx-3 association as a potential target for therapeutic intervention and suggest that it might influence the progression of spinocerebellar ataxia type 3.

URL for the Abstract:

http://www.nature.com/emboj/journal/v25/n7/abs/7601043a.html

Categories,
Keywords:

ataxin-3, VCP, polyglutamine aggregation

HyperLinks / References / URLs:


Copyright Message:


Personal Comments:


Download
Access Level:

Intranet

Correlation

MPG Unit:

Max-Planck-Institut für Informatik



MPG Subunit:

Computational Biology and Applied Algorithmics

Appearance:

MPII WWW Server, MPII FTP Server, MPG publications list, university publications list, working group publication list, Fachbeirat, VG Wort


BibTeX Entry:

@ARTICLE{Albrecht2006e,
AUTHOR = {Boeddrich, Annett and Gaumer, S{\'e}bastien and Haacke, Annette and Tzvetkov, Nikolay and Albrecht, Mario and Evert, Bernd O. and M{\"u}ller, Eva C. and Lurz, Rudi and Breuer, Peter and Schugardt, Nancy and Plaßmann, Stephanie and Xu, Kexiang and Warrick, John M. and Suopanki, Jaana and W{\"u}llner, Ullrich and Frank, Ronald and Hartl, Ulrich F. and Bonini, Nancy M. and Wanker, Erich E.},
TITLE = {An arginine/lysine-rich motif is crucial for {VCP/p97}-mediated modulation of ataxin-3 fibrillogenesis},
JOURNAL = {EMBO Journal},
YEAR = {2006},
NUMBER = {7},
VOLUME = {25},
PAGES = {1547--1558},
}


Entry last modified by Uwe Brahm, 07/11/2007
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Editor(s)
Mario Albrecht
Created
03/23/2006 07:54:23 PM
Revisions
4.
3.
2.
1.
0.
Editor(s)
Uwe Brahm
Christine Kiesel
Christine Kiesel
Mario Albrecht
Mario Albrecht
Edit Dates
2007-07-11 12:30:23
20.02.2007 16:46:04
20.02.2007 16:40:44
23.05.2006 20:08:25
23.03.2006 19:54:23