Article
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.
The Journal of biological chemistry - 16 Aug 2002
Mattiazzi Marina, D'Aurelio Marilena, Gajewski Carl D, Martushova Katherine, Kiaei Mahmoud, Beal M Flint, Manfredi Giovanni
Abstract excerpt
A growing body of evidence suggests that impaired mitochondrial energy production and increased oxidative radical damage to the mitochondria could be causally involved in motor neuron death in amyotrophic lateral sclerosis (ALS) and in familial ALS associated with mutations of Cu,Zn superoxide dismutase (SOD1). For example, morphologically abnormal mitochondria and impaired mitochondrial histoenzymatic...
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