Name: U-Blot® SOD1 Rabbit mAb
SKU: WR4045
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 inThe 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

Size	50μL/100μL
Target	SOD-1
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, ELISA
MW(Calculated)	16kD
MW(Observed)	15kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:100-1:5000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM0113R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	SOD1
Protein Name	Superoxide dismutase [Cu-Zn]
Other Name	SOD1; / Superoxide dismutase [Cu-Zn]; / Superoxide dismutase 1; / hSod1
Species	Human
Gene ID	6647
Uniprot	P00441,
Species.1	Mouse
Gene ID.1	20655
Uniprot.1	P08228,
Species.2	Rat
Gene ID.2	24786
Uniprot.2	P07632
Cellular Localization	Cytoplasm, Nucleus
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.,

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U-Blot® SOD1 Rabbit mAb

Product details

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U-Blot® SOD1 Rabbit mAb

Product details

Specifications

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