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| Gene Name | Description |
|---|---|
| ACO1 | Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; mutation in human homolog ACO2 is associated with dominant optic nerve atrophy; human homolog ACO2 can complement yeast null mutant |