OPA1 deficiency associated with increased autophagy in retinal ganglion cells in a murine model of dominant optic atrophy.

@article{White2009OPA1DA,
  title={OPA1 deficiency associated with increased autophagy in retinal ganglion cells in a murine model of dominant optic atrophy.},
  author={Kathryn Elizabeth White and Vanessa J Davies and Vanessa E. Hogan and Malgorzata J Piechota and Philip P Nichols and Douglas M. Turnbull and Marcela Votruba},
  journal={Investigative ophthalmology & visual science},
  year={2009},
  volume={50 6},
  pages={2567-71}
}
PURPOSE To examine retinal ganglion cell (RGC) and axonal abnormalities in an ENU-induced mutant mouse carrying a protein-truncating nonsense mutation in OPA1. Mutations in the OPA1 gene cause autosomal dominant optic atrophy (ADOA) in which loss of RGCs followed by myelin degeneration in the optic nerve leads to progressive decrease in visual acuity. METHODS Ultrastructure of the optic nerve was examined in heterozygous mutants and wild-type littermate controls at 6, 9, and 24 months using… CONTINUE READING

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