Overview:

SOD2 (superoxide dismutase 2) is a member of the iron/manganese superoxide dismutase family. SOD2 binds to the superoxide byproducts of the mitochondrial electron transport chain and converts them to hydrogen peroxide and diatomic oxygen. Failure of SOD2 to remove the superoxide byproducts leads to an increase in mitochondrial reactive oxygen species resulting in biochemical aberrations with features reminiscent of mitochondrial myopathy, Friedreich ataxia, and HMGCL deficiency (1). Mutations in SOD2 have been associated with idiopathic cardiomyopathy (IDC), premature aging, sporadic motor neuron disease, and cancer (2).

Gene Aliases:

Mn-SOD, MNSOD, IPO-B, Superoxide Dismutase 2

Genbank Number:

NM_000636

References:

1. Melov, S. et al: A novel neurological phenotype in mice lacking mitochondrial manganese superoxide dismutase. Nature Genet. 18: 159-163, 1998.

2. Hiroi, S. et al: Polymorphisms in the SOD2 and HLA-DRB1 genes are associated with nonfamilial idiopathic dilated cardiomyopathy in Japanese. Biochem. Biophys. Res. Commun. 261: 332-339, 1999.