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Cell damage is induced by reactive oxygen species (ROS). ROS are either free radicals, reactive anions
containing oxygen atoms, or molecules containing oxygen atoms that can either produce free radicals or are
chemically activated by them. Examples are hydroxyl radical, superoxide, hydrogen peroxide, and peroxynitrite.
The main source of ROS in vivo is aerobic respiration, although ROS are also produced by peroxisomal oxidation of fatty acids, microsomal cytochrome P450 metabolism of xenobiotic compounds, stimulation of
phagocytosis by pathogens or lipopolysaccharides, arginine metabolism, and tissue specific enzymes. Under
normal conditions, ROS are cleared from the cell by the action of superoxide dismutase (SOD), catalase, or
glutathione (GSH) peroxidase. The main damage to cells results from the ROS-induced alteration of
macromolecules such as polyunsaturated fatty acids in membrane lipids, essential proteins, and DNA.
Additionally, oxidative stress and ROS have been implicated in disease states, such as Alzheimer's disease,
Parkinson's disease, cancer, and aging. Superoxide Dismutase (SOD) catalyzes the reduction of superoxide
anions to hydrogen peroxide. KM for O2 for bovine SOD = 0.35 mM. The bovine enzyme exists as a dimeric
copper-zinc containing protein with a molecular weight of 2 X 16,300 Da. The E. coli enzyme exists as a dimeric
manganese or iron containing glycoprotein with a molecular weight of 2 x 22,000 Da. The human enzyme exists
as a tetrameric copper-zinc or manganese containing glycoprotein with a molecular weight of 2 X 2328,300 Da.
Covalent conjugation of superoxide dismutase with polyethylene glycol (PEG) has been found to increase the
circulatory half-life and provides prolonged protection from partially reduced oxygen species.
http://www.ncbi.nlm.nih.gov/gene?cmd=search&term=6647
Also known as
ALS; SOD; ALS1; IPOA; hSod1; homodimer
Summary
The protein encoded by this gene binds copper and zinc ions and is one of two isozymes
responsible for destroying free superoxide radicals in the body. The encoded 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. [provided by RefSeq, Jul
2008]
Location :
21q22.11
Sequence :
Chromosome: 21; NC_000021.8 (33031935..33041244)
See SOD1 in Epigenomics, MapViewer
Chromosome 21 - NC_000021.8
http://www.ncbi.nlm.nih.gov/gene?cmd=search&term=6648
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