Pathophysiology of the optic neuropathy associated with Friedreich ataxia.

@article{Alldredge2003PathophysiologyOT,
  title={Pathophysiology of the optic neuropathy associated with Friedreich ataxia.},
  author={Claron D Alldredge and Christopher R Schlieve and N. R. Miller and Leonard A Levin},
  journal={Archives of ophthalmology},
  year={2003},
  volume={121 11},
  pages={
          1582-5
        }
}
OBJECTIVES To describe the optic neuropathy associated with the genetic defect in Friedreich ataxia and suggest a pathophysiologic mechanism. METHODS An experimental model of retinal ganglion cell death in the presence of metal chelation was used to test a hypothetical mechanism for the optic neuropathy of Friedreich ataxia. RESULTS Study of cultured rat retinal ganglion cells suggests that abnormal regulation of intracellular iron levels could increase sensitivity to reactive oxygen… CONTINUE READING
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