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dominant optic atrophy

Friday 27 January 2006

Etiology

OPA1 is caused by defects in a dynamin-related protein that is targeted to mitochondria and might exert its function in mitochondrial biogenesis and in stabilization of mitochondrial membrane complexes.

References

- Shoubridge EA. Nuclear genetic defects of oxidative phosphorylation. Hum Mol Genet. 2001 Oct 1;10(20):2277-84. PMID: 11673411

- Smeitink J, van den Heuvel L, DiMauro S. The genetics and pathology of oxidative phosphorylation. Nat Rev Genet. 2001 May;2(5):342-52. PMID: 11331900