[HTML][HTML] Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene
G Manfredi, N Gupta, ME Vazquez-Memije… - Journal of Biological …, 1999 - ASBMB
AT→ G mutation at position 8993 in human mitochondrial DNA is associated with the
syndrome neuropathy, ataxia, and retinitis pigmentosa and with a maternally inherited form
of Leigh's syndrome. The mutation substitutes an arginine for a leucine at amino acid
position 156 in ATPase 6, a component of the F 0 portion of the mitochondrial ATP synthase
complex. Fibroblasts harboring high levels of the T8993G mutation have decreased ATP
synthesis activity, but do not display any growth defect under standard culture conditions …
syndrome neuropathy, ataxia, and retinitis pigmentosa and with a maternally inherited form
of Leigh's syndrome. The mutation substitutes an arginine for a leucine at amino acid
position 156 in ATPase 6, a component of the F 0 portion of the mitochondrial ATP synthase
complex. Fibroblasts harboring high levels of the T8993G mutation have decreased ATP
synthesis activity, but do not display any growth defect under standard culture conditions …