Anti-Superoxide Dismutase 1

P00441

FUNCTION: SwissProt: P00441 # Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
COFACTOR: Binds 1 copper ion per subunit. & Binds 1 zinc ion per subunit.
SIZE: 154 amino acids; 15936 Da
SUBUNIT: Homodimer. The pathogenics variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not.
SUBCELLULAR LOCATION: Cytoplasm.
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.
DISEASE: SwissProt: P00441 # 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: SwissProt: P00441 ## Belongs to the Cu-Zn superoxide dismutase family.
MISCELLANEOUS: Zinc binding promotes dimerization. ... nascondi » visualizza altro »

NM_000454.4

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. ... nascondi » visualizza altro »

Anti-Superoxide Dismutase 1

Mutations in the copper/ zinc superoxide dismutase (SOD1) gene are associated with 15- 20% of the familial forms of motoneuron disease. This 153 amino acid metalloenzyme is expressed in virtually all cells of all organisms above bacteria and is highly conserved across species, although some minor variations do occur. The incorporation of the mutated form of the SOD1 human gene into a transgenic mouse leads to the onset of the disease that closely resembles the human condition. These animals become weak at about 2-4 months of age and rapidly lose function, which results in death 4- 6 weeks later.

SOD1

Superoxide Dismutase 1 (SOD1), mouse and rat specific. No reactivity with human SOD1

Western Blotting

Western blotting: 1:200-1:500 (see images)

Optimal working dilutions must be determined by end user.

• Mouse
• Rat

Human

Synthetic peptide corresponding to amino acids 25-37 of mouse SOD1.

Rabbit serum. Lyophilized. Contains no preservative. Reconstitute with 50 μL of sterile water.

Maintain lyophilized material at -70°C (dry) for up to 12 months after date of receipt. After reconstitution maintain frozen at -20°C in undiluted aliquots for up to 6 months. Avoid repeated freeze/thaw cycles. Glycerol (1:1) can be added for additional stability.

• SOD1
• ALS1
• IPOA
• Cu-Zn
• SOD
• ALS
• homodimer
• EC 1.15.1.1

Neuroscience

Oxidative Stress

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Chemicon (Millipore)

Serum

Rabbit

Polyclonal Antibody