Anti-Superoxide dismutase type 1 Antibody, Non-Fucosylated (BioBet-1306ZP) (CAT#: BioBet-1306ZP) Datasheet

SOD1

superoxide dismutase 1, soluble

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.

SOD1; superoxide dismutase 1, soluble; ALS; SOD; ALS1; IPOA; hSod1; HEL-S-44; homodimer; superoxide dismutase [Cu-Zn]; SOD, soluble; indophenoloxidase A; Cu/Zn superoxide dismutase; superoxide dismutase, cystolic; epididymis secretory protein Li 44

Mitochondrion, Nucleus

Diseases associated with SOD1 include Amyotrophic Lateral Sclerosis 1 and Spastic Tetraplegia And Axial Hypotonia, Progressive.

Its related pathways are Neuroscience and Association Between Physico-Chemical Features and Toxicity Associated Pathways.

Destroys radicals which are normally produced within the cells and which are toxic to biological systems.

Cancer antibody; Cell Biology and Cellular Response antibody; Cell Death antibody; Gene Regulation antibody; Metabolism antibody; Microbiology and Infectious Disease antibody; Neuroscience antibody; Signaling Transduction antibody

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. Palmitoylation helps nuclear targeting and decreases catalytic activity. Succinylation, adjacent to copper catalytic site, probably inhibits activity. Desuccinylation by SIRT5 enhances activity.

Protein Based Therapies
Monoclonal antibody (mAb)

IgG

BioBet-1306ZP

Humanized

Human

The anti-SOD1 antibody sre used for the treatment of amyotrophic lateral sclerosis (ALS) or the amelioration of symptoms associated with ALS.

Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis