Product Name	SOD1
Chinese Name	超氧化物歧化酶1（铜/锌过氧化物歧化酶SOD）Recombinant rabbit monoclonal anti
Alias	Superoxide Dismutase 1; ALS 1; ALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Amyotrophic lateral sclerosis 1; Cu/Zn SOD; Cu/Zn superoxide dismutase; Homodimer; Indophenoloxidase A; IPOA; Mn superoxide dismutase; SOD 1; SOD; SOD soluble;SOD2; SODC; Soluble indophenoloxidase A; Superoxide dismutase 1; Superoxide dismutase 1 soluble; Superoxide dismutase Cu Zn; Superoxide dismutase cystolic; SODC_HUMAN; Superoxide dismutase [Cu-Zn]; hSod1; Ipo1; SODC; Ipo-1; Sod-1; CuZnSOD; Cu/Zn-SOD; MGC107553; B430204E11Rik; superoxide-dimutase-1.
Immunogen Species	Rabbit
Clonality	Monoclonal
React Species	(predicted: Human, Mouse, Rat, )
Applications	not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	17kDa
Cellular localization	The nucleus cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human SOD1
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	PBS, Glycerol, BSA
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] Function: Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Subunit: Homodimer; non-disulfide linked. Homodimerization may take place via the ditryptophan cross-link at Trp-33. The pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not. The pathogenic variants ALS1 Arg-86 and Ala-94 interact with MARCH5, whereas wild-type protein does not. Subcellular Location: Cytoplasm. Note=The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. Post-translational modifications: Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation. The ditryptophan cross-link at Trp-33 is responsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required. DISEASE: Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) [MIM:105400]. ALS1 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms. Similarity: Belongs to the Cu-Zn superoxide dismutase family. SWISS: P00441 Gene ID: 6647 Database links: Entrez Gene: 6647 Human Entrez Gene: 20655 Mouse Entrez Gene: 24786 Rat Omim: 147450 Human SwissProt: P00441 Human SwissProt: P08228 Mouse SwissProt: P07632 Rat Unigene: 443914 Human Unigene: 276325 Mouse Unigene: 466779 Mouse Unigene: 6059 Rat 超氧化物歧化酶又称铜/锌过氧化物歧化酶SOD(Superoxide dismutase，简称SOD)是参与机体抗氧化（ROS，反应性氧离子reactive oxygen species）防御机制和抵御细胞氧化损伤最重要的酶类之一， 广泛存在于需氧生物、耐氧生物及某些厌氧微生物中，目前已知的SOD 主要分为三类，即胞质中Cu/Zn-SOD（即SOD1）、Mitochondrion中的Mn-SOD（即SOD2）和ec-SOD(即SOD3)。 超氧化物歧化酶-1SOD1的水平与很多生理反应有关，如：应急，热休克，紫外和X线照射等。SOD1水平降低能触发AP2转录因子的激活。SOD1在临床上对很多疾病诊断有重要意义。

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