The BiP molecular chaperone plays multiple roles during the biogenesis of TorsinA, an AAA+ ATPase associated with the neurological disease Early-Onset Torsion Dystonia

Zacchi, Lucía F., Wu, Hui-Chuan, Bell, Samantha L., Millen, Linda, Paton, Adrienne W., Paton, James C., Thomas, Philip J., Zolkiewski, Michal and Brodsky, Jeffrey L. (2014) The BiP molecular chaperone plays multiple roles during the biogenesis of TorsinA, an AAA+ ATPase associated with the neurological disease Early-Onset Torsion Dystonia. Journal of Biological Chemistry, 289 18: 12727-12747. doi:10.1074/jbc.M113.529123

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Author Zacchi, Lucía F.
Wu, Hui-Chuan
Bell, Samantha L.
Millen, Linda
Paton, Adrienne W.
Paton, James C.
Thomas, Philip J.
Zolkiewski, Michal
Brodsky, Jeffrey L.
Title The BiP molecular chaperone plays multiple roles during the biogenesis of TorsinA, an AAA+ ATPase associated with the neurological disease Early-Onset Torsion Dystonia
Journal name Journal of Biological Chemistry   Check publisher's open access policy
ISSN 0021-9258
1083-351X
Publication date 2014
Sub-type Article (original research)
DOI 10.1074/jbc.M113.529123
Open Access Status File (Publisher version)
Volume 289
Issue 18
Start page 12727
End page 12747
Total pages 21
Place of publication Bethesda, MD, United States
Publisher American Society for Biochemistry and Molecular Biology
Language eng
Formatted abstract
Background: The E mutation in the AAA ATPase torsinA is associated with the neurological disease torsion dystonia.

Results: BiP and its co-factors maintain torsinA and torsinAE stability, glycosylation, and solubility.

Conclusion: torsinA/E, a chaperone-like protein, requires the assistance of other chaperones to fold.

Significance: Therapeutics that modulate BiP may counteract torsinAE-associated physiological defects.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Chemistry and Molecular Biosciences
 
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Created: Mon, 11 Apr 2016, 23:05:15 EST by Lucia Zacchi on behalf of School of Chemistry & Molecular Biosciences