Leigh disease caused by the mitochondrial DNA G14459A mutation in unrelated families.

Kirby, D M; Kahler, S G; Freckmann, M L; Reddihough, D; Thorburn, D R · Ann Neurol · 2000

case_series · Level IV

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Abstract

Leigh disease can be caused by defects of both nuclear and mitochondrially encoded genes. One mitochondrial DNA mutation, G14459A, has been associated with both respiratory chain complex I deficiency and Leber's hereditary optic neuropathy, with or without dystonia. Here, we report the occurrence of this mutation in 3 complex I-deficient patients from 2 separate pedigrees who presented with Leigh disease, with no evidence or family history of Leber's hereditary optic neuropathy or dystonia.

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