Journal article
Exploring the Influence of TorsinA Expression on Protein Quality Control
Neurochemical research, Vol.36(3), pp.452-459
03/2011
DOI: 10.1007/s11064-010-0363-4
PMID: 21161590
Abstract
DYT1 dystonia is caused by a glutamic acid deletion (ΔE) in the endoplasmic reticulum (ER) protein torsinA. Previous studies suggest that torsinA modulates the aggregation of cytosolic misfolded proteins and ER stress responses, although the mechanisms underlying those effects remain unclear. In order to investigate the bases of these observations, we analyzed the interaction between torsinA expression, protein aggregation and ER stress in PC6.3 cells. Unexpectedly, we found that expression of torsinA(wt) or (ΔE) does not influence the inclusion formation by an expanded polyglutamine reporter protein in this cellular model. Furthermore, torsinA does not prevent the activation of ER stress induced by thapsigargin or the reducing agent DTT. Interestingly, DTT induces post-translational changes in torsinA, more prominently for torsinA(wt) than (ΔE). This work highlights the importance of model system selection for the study of torsinA function. Furthermore, it provides additional evidence suggesting that torsinA is sensitive to changes in the cellular redox potential.
Details
- Title: Subtitle
- Exploring the Influence of TorsinA Expression on Protein Quality Control
- Creators
- Kara Gordon - Graduate Program in Neuroscience The University of Iowa Iowa City IA USAKevin Glenn - Department of Internal Medicine The University of Iowa Iowa City IA 52242 USAPedro Gonzalez-Alegre - Department of Neurology, Carver College of Medicine The University of Iowa 3111A MERF, 375 Newton Road Iowa City IA 52242 USA
- Resource Type
- Journal article
- Publication Details
- Neurochemical research, Vol.36(3), pp.452-459
- Publisher
- Springer US
- DOI
- 10.1007/s11064-010-0363-4
- PMID
- 21161590
- ISSN
- 0364-3190
- eISSN
- 1573-6903
- Language
- English
- Date published
- 03/2011
- Academic Unit
- General Internal Medicine; Internal Medicine
- Record Identifier
- 9984094594702771
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