Premature oxidative aging of hepatic mitochondrial DNA in Wilson's disease
A Mansouri, I Gaou, B Fromenty, A Berson… - …, 1997 - gastrojournal.org
BACKGROUND & AIMS: Aging is associated with and may be caused by acquired somatic
mutations of the mitochondrial genome. In Wilson's disease, inherited mutations of a nuclear
gene encoding a copper transporter cause accumulation of copper in the liver, particularly
within mitochondria. Because copper has prooxidant properties and the mitochondrial
genome is particularly susceptible to oxidative damage, we hypothesized that Wilson's
disease may cause premature oxidative aging of mitochondrial DNA. METHODS: Hepatic …
mutations of the mitochondrial genome. In Wilson's disease, inherited mutations of a nuclear
gene encoding a copper transporter cause accumulation of copper in the liver, particularly
within mitochondria. Because copper has prooxidant properties and the mitochondrial
genome is particularly susceptible to oxidative damage, we hypothesized that Wilson's
disease may cause premature oxidative aging of mitochondrial DNA. METHODS: Hepatic …