Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mutant SOD1.

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.

Cell culture models to investigate the selective vulnerability of motoneuronal mitochondria to familial ALS-linked G93ASOD1.

Review Mitochondrial dysfunction in familial amyotrophic lateral sclerosis.

Review Transgenic mouse model for familial amyotrophic lateral sclerosis with superoxide dismutase-1 mutation.

Review Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.

Amyotrophic lateral sclerosis-associated mutant SOD1 inhibits anterograde axonal transport of mitochondria by reducing Miro1 levels.

A Cystine-Rich Whey Supplement (Immunocal®) Provides Neuroprotection from Diverse Oxidative Stress-Inducing Agents <i>In Vitro</i> by Preserving Cellular Glutathione.