Antibody is stable for 24 months.
Positive Control: HeLa or Jurkat cells. Human breast or ovarian carcinoma Cellular Localization: Cytoplasm
Specificity Comments: Cu-Zn superoxide dismutase-1 (SOD-1) is a well-characterized cytosolic scavenger of oxygen free radicals that requires copper and zinc binding to potentiate its enzymatic activity. Enzymatically, SOD-1 facilitates the dismutation of oxygen radicals to hydrogen peroxide and also catalyzes pro-oxidant reactions, which include the peroxidase activity and hydroxyl radical generating activity. SOD-1 is ubiquitously expressed in somatic cells and functions as a homodimer. Defects in the gene encoding SOD-1 have been implicated in the progression of neurological diseases, including amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by the loss of spinal motor neurons, Down syndrome and Alzheimer s disease. In familial ALS, several mutations in SOD-1 predominate, resulting in the loss of zinc binding, the loss of scavenging activity of SOD-1, and correlate with an increase in neurotoxicity and motor neuron death.
SOD1 (superoxide dismutase) binds copper and zinc ions and is one of two isozymes responsible for destroying free superoxide radicals in the body. This isozyme is a soluble cytoplasmic protein, acting as a homodimer to convert naturally-occuring but harmful superoxide radicals to molecular oxygen and hydrogen peroxide. The other isozyme is a mitochondrial protein. Mutations in this gene have been implicated as causes of familial amyotrophic lateral sclerosis. Rare transcript variants have been reported for this gene.
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Protein Aliases: Amyotrophic lateral sclerosis 1 (ALS1); Cu/Zn SOD; Cu/Zn superoxide dismutase; epididymis secretory protein Li 44; HGNC:11179; hSod1; indophenoloxidase A; Indophenoloxidase A (IPOA); SOD, soluble; superoxide dismutase; Superoxide dismutase 1; Superoxide Dismutase 1 (SOD1); Superoxide dismutase [Cu-Zn]; superoxide dismutase, cystolic
Gene Aliases: ALS; ALS1; HEL-S-44; homodimer; hSod1; IPOA; SOD; SOD1
UniProt ID: (Human) P00441
Entrez Gene ID: (Human) 6647
Molecular Function: oxidoreductase