Superoxide Dismutase / SOD1抗体

Superoxide Dismutase / SOD1抗体产品信息免疫原 : Recombinant human SOD1 protein ( Catalog#11727-H07E )

Antibody Type : Rabbit Monoclonal Antibody ( Rabbit mAb Service Platform )
克隆号 : 101
抗体宿主 : Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 : This antibody was obtained from a rabbit immunized with purified, human cell-derived, recombinant human SOD1 ( rh SOD1 ; Catalog#11727-H07E ; NP_000445.1 ; Ala 2 - Gln 154 ).
Superoxide Dismutase / SOD1抗体 Background
Superoxide dismutase [Cu-Zn], also known as superoxide dismutase 1 and SOD1, is an enzyme that in humans is a cytoplasm protein which belongs to the Cu-Zn superoxide dismutase family. SOD1 binds copper and zinc ions and is one of three isozymes responsible for destroying free superoxide radicals in the body. SOD1 is a soluble cytoplasmic and mitochondrial intermembrane space protein, acting as a homodimer to convert naturally occurring, but harmful, superoxide radicals to molecular oxygen and hydrogen peroxide. SOD1 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. Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) which is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Death usually occurs within 2 to 5 years. The disease is inherited in 5-10% of cases leading to familial forms.
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