Name: SOD-1 Monoclonal Antibody
SKU: WM0426
Price: 268 USD
Availability: InStock

Product details

Background:
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],

Specifications

Target	SOD-1
Reactivity	Human, Mouse
Applications	WB, IF, FC, ELISA
MW(Calculated)	16kD
MW(Observed)
Host Species	Mouse
Isotype
Conjugate/Modification	Unmodified
Modification
Recommended Dilution Ratio	WB 1:500-1:2000; IF 1:200-1:1000; Flow Cyt 1:200-1:400; ELISA 1:10000; Not yet tested in other applications.
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Source
Purification	Affinity purification
Purity
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	5D12
Immunogen	Purified recombinant fragment of human SOD1 expressed in E. Coli.
Sequence
Specificity	SOD1 Monoclonal Antibody detects endogenous levels of SOD1 protein.
Gene Name	SOD1
Protein Name	Superoxide dismutase [Cu-Zn]
Other Name	SOD1; / Superoxide dismutase [Cu-Zn]; / Superoxide dismutase 1; / hSod1
Species	Human
Gene ID-1	6647
Uniprot	P00441,
Species.1	Mouse
Gene ID-2	20655
Uniprot.1	P08228
Species.2
Gene ID-3
Uniprot.2
Organism-4
Gene ID-4
SwissProt-4
Background	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],
Cellular Localization	Cytoplasm . Mitochondrion . Nucleus . Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. .
Tissue Expression	Colon,Fetal brain cortex,Placenta,
Signaling_pathway	Cellular Processes >> Transport and catabolism >> Peroxisome;Organismal Systems >> Aging >> Longevity regulating pathway - multiple species;Human Diseases >> Neurodegenerative disease >> Parkinson disease;Human Diseases >> Neurodegenerative disease >> Amyotrophic lateral sclerosis;Human Diseases >> Neurodegenerative disease >> Huntington disease;Human Diseases >> Neurodegenerative disease >> Prion disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>Peroxisome; / >>Longevity regulating pathway - multiple species; / >>Parkinson disease; / >>Amyotrophic lateral sclerosis; / >>Huntington disease; / >>Prion disease; / >>Pathways of neurodegeneration - multiple diseases; / >>Chemical carcinogenesis - reactive oxygen species
Function	Catalytic activity:2 superoxide + 2 H(+) = O(2) + H(2)O(2).,cofactor:Binds 1 copper ion per subunit.,cofactor:Binds 1 zinc ion per subunit.,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.,Function:Destroys radicals which are normally produced within the cells and which are toxic to biological systems.,miscellaneous:The protein (both wild-type and ALS1 variants) has a tendency to form fibrillar aggregates in the absence of the intramolecular disulfide bond or of bound zinc ions. These aggregates may have cytotoxic effects. Zinc binding promotes dimerization and stabilizes the native form.,online information:ALS genetic mutations db,online information:Superoxide dismutase entry,PTM:Unlike wild-type protein, the pathogenics variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A; which leads to their proteasomal degradation.,similarity:Belongs to the Cu-Zn superoxide dismutase family.,subunit:Homodimer. The pathogenics variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not.,
RRID

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SOD-1 Monoclonal Antibody

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SOD-1 Monoclonal Antibody

Product details

Specifications

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