mt-Nd6^m3Dwa

Treatment with psoralen and UV light was used to generate a clone (LT13) homoplastic for mitochondria with a G-to-A mutation at position 13997 that results in the amino acid substitution of proline with leucine at position 25 (p.P25L). This mutation is equivalent to the human mutation g.14600G>A (p.P25L) associated with optic atrophy and Leigh syndrome. Rhodamine-6G treatment was used to deplete ES cells of endogenous mitochondria. (J:192260)