Aprataxin localizes to mitochondria and preserves mitochondrial function.

  title={Aprataxin localizes to mitochondria and preserves mitochondrial function.},
  author={Peter Sykora and Deborah L. Croteau and Vilhelm A. Bohr and David M Wilson},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={108 18},
Ataxia with oculomotor apraxia 1 is caused by mutation in the APTX gene, which encodes the DNA strand-break repair protein aprataxin. Aprataxin exhibits homology to the histidine triad superfamily of nucleotide hydrolases and transferases and removes 5'-adenylate groups from DNA that arise from aborted ligation reactions. We report herein that aprataxin localizes to mitochondria in human cells and we identify an N-terminal amino acid sequence that targets certain isoforms of the protein to this… CONTINUE READING