Overexpression of F0F1-ATP synthase α suppresses mutant huntingtin aggregation and toxicity in vitro

Hong Quan Wang, Yu Xia Xu, Xiao Yan Zhao, Hong Zhao, Jie Yan, Xiao Bo Sun, Jing Chun Guo, Cui Qing Zhu

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Huntington's disease (HD) and other polyglutamine (polyQ) neurodegenerative diseases are characterized by neuronal accumulation of the disease protein, suggesting that the cellular ability to handle abnormal proteins is compromised. As a multi-subunit protein localized in the mitochondria of eukaryotic cells, the F0F1-ATP synthase α belongs to the family of stress proteins HSP60. Currently, mounting evidences indicate F0F1-ATP synthase α may play a role in neurodegenerative diseases, including Alzheimer's disease (AD) and Parkinson's disease (PD). Recently, ATP synthase α was reported to have protective and therapeutic roles in primary cardiacmyocytes of iron-overloaded rats by lowering ROS production. However, little is understood about the role of ATP synthase α in cell death and neurodegeneration. Here, we demonstrate that overexpression of ATP synthase α suppresses huntingtin (htt) polyQ aggregation and toxicity in transfected SH-SY5Y cell lines. Overexpression of ATP synthase α is able to protect cell death caused by polyglutamine-expanded htt. Transient overexpression of ATP synthase α suppresses the aggregate formation by estimation of polyQ aggregation, Western blot analysis, and filter trap assay (FTA) in transfected SH-SY5Y cells. These results indicated that ATP synthase α has a strong inhibitory effect on polyglutamine aggregate formation and toxicity in vitro, and suggest a novel neuroprotective role of ATP synthase α.

Original languageEnglish (US)
Pages (from-to)1294-1298
Number of pages5
JournalBiochemical and Biophysical Research Communications
Issue number4
StatePublished - Dec 25 2009


  • ATP synthase α
  • HSP
  • Huntingtin
  • Overexpression

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Cell Biology
  • Molecular Biology


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