Although reactive oxygen species are natural metabolic products, they
can be toxic to cells and are implicated in some neurodegenerative dis
eases. Cytosolic Cu,Zn superoxide dismutase normally defends against d
amage by reactive oxygen species; however, mutant forms of the enzyme
might instead contribute to damage of motor neurons in some amyotrophi
c lateral sclerosis patients. Possible mechanisms of oxidative injury
to neurons are discussed with reference to cytosolic Cu,Zn superoxide
dismutase mutations and other factors which might enhance oxygen radic
al toxicity.