ABSTRACT
Objective To study the genetic origin of mitochondrial dysfunction in patients with mitochondrial myopathy.Methods The esphagus carcinoma cells were cultured by ethidium bromide, and established stabile,cell line of long term survival mitochondrial DNA(mtDNA).The platelets of patients with mitochondrial myopathy and the normal controls were carried out cell fusion.the mitochondrial function of fusion cell was determined. Results The esphagus carcinoma cells were cultured by ethidium bromide for 12 days, the cells were completely depleted of mtDNA,which can be passed stably. The respiratory capacity of transformants derived from patients with mitochondrial myopathy was lower than those from the control.Conclusion The mtDNA mutation can play a role in the pathogenesis of mitochondrial myopathy.