Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function.

@article{Davies2007Opa1DI,
  title={Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function.},
  author={Vanessa J Davies and Andrew J. Hollins and Malgorzata J Piechota and W. San Yip and Jennifer Davies and Kathryn Elizabeth White and Phillip P Nicols and Michael Boulton and Marcela Votruba},
  journal={Human molecular genetics},
  year={2007},
  volume={16 11},
  pages={1307-18}
}
OPA1 is a ubiquitously expressed, nuclear dynamin-related GTPase, targeted to the inner mitochondrial membrane, which plays a role in mitochondrial fusion. Mutations in the OPA1 gene on chromosome 3q28-qter are associated with autosomal dominant optic atrophy (ADOA), the most common inherited optic neuropathy, in which retinal ganglion cells (RGCs) are lost and visual acuity is impaired from an early age. We have generated a novel ENU-induced mutant mouse carrying a protein-truncating nonsense… CONTINUE READING

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