Overexpression of torsinA in PC12 cells protects against toxicity

P. Shashidharan, Nicolae Paris, Daniela Sandu, Laina Karthikeyan, Kevin St P. McNaught, Ruth H. Walker, C. Warren Olanow

Research output: Contribution to journalArticlepeer-review

43 Scopus citations


Childhood-onset dystonia is an autosomal dominant movement disorder associated with a three base pair (GAG) deletion mutation in the DYT1 gene. This gene encodes a novel ATP-binding protein called torsinA, which in the central nervous system is expressed exclusively in neurons. Neither the function of torsinA nor its role in the pathophysiology of DYT1 dystonia is known. In order to better understand the cellular functions of torsinA, we established PC12 cell lines overexpressing wild-type or mutant torsinA and subjected them to various conditions deleterious to cell survival. Treatment of control PC12 cells with an inhibitor of proteasomal activity, an oxidizing agent, or trophic withdrawal, resulted in cell death, whereas PC12 cells that overexpressed torsinA were significantly protected against each of these treatments. Overexpression of mutant torsinA failed to protect cells against trophic withdrawal. These results suggest that torsinA may play a protective role in neurons against a variety of cellular insults.

Original languageEnglish
Pages (from-to)1019-1025
Number of pages7
JournalJournal of Neurochemistry
Issue number4
StatePublished - Feb 2004


  • Dystonia
  • Neuroprotection
  • Oxidative stress
  • Proteasome
  • TorsinA
  • Trophic withdrawal


Dive into the research topics of 'Overexpression of torsinA in PC12 cells protects against toxicity'. Together they form a unique fingerprint.

Cite this