The bip molecular chaperone plays multiple roles during the biogenesis of torsina, an aaa atpase associated with the neurological disease early-onset torsion dystonia

Lucía F. Zacchi, Hui Chuan Wu, Samantha L. Bell, Linda Millen, Adrienne W. Paton, James C. Paton, Philip J. Thomas, Michal Zolkiewski, Jeffrey L. Brodsky

Research output: Contribution to journalArticle

13 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)12727-12747
Number of pages21
JournalJournal of Biological Chemistry
Volume289
Issue number18
DOIs
StatePublished - 2014

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Dystonia Musculorum Deformans
Glycosylation
Molecular Chaperones
Torsional stress
Solubility
Adenosine Triphosphatases
Defects
Mutation
Proteins
Therapeutics
Dystonia musculorum deformans type 1

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Cite this

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; Brodsky, Jeffrey L.

In: Journal of Biological Chemistry, Vol. 289, No. 18, 2014, p. 12727-12747.

Research output: Contribution to journalArticle

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. / The bip molecular chaperone plays multiple roles during the biogenesis of torsina, an aaa atpase associated with the neurological disease early-onset torsion dystonia. In: Journal of Biological Chemistry. 2014 ; Vol. 289, No. 18. pp. 12727-12747.
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AU - Bell, Samantha L.

AU - Millen, Linda

AU - Paton, Adrienne W.

AU - Paton, James C.

AU - Thomas, Philip J.

AU - Zolkiewski, Michal

AU - Brodsky, Jeffrey L.

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