Creation and characterization of mitochondrial DNA-depleted cell lines with neuronal-like properties

摘要: Mitochondrial dysfunction and attendant bioenergetic defects are increasingly recognized as playing an important role in neurodegenerative disorders. The increased attention on mitochondrial involvement points to the need for developing cell lines that have neuron-like characteristics genetic analysis modeling of these diseases. We describe creation respiratory-deficient SH-SY5Y neuroblastoma (rho zero 64/5) by selectively depleting DNA through prolonged exposure ethidium bromide. Oxygen consumption cells activities electron transport chain enzyme complexes I IV contain subunits encoded genome eliminated. In contrast, function complex II, a nuclear-encoded component, is largely intact cells. rho 64/5 retain ability differentiate into with phenotypes following treatment phorbol ester or retinoic acid. Normal respiratory recovered repopulation exogenous human platelet mitochondria. line serves valuable model study neurologic diseases suspected involving dysfunction.