Recombinant Human Superoxide dismutase [Cu-Zn] protein (SOD1) (Active)

Recombinant Human Superoxide dismutase [Cu-Zn] protein (SOD1) (Active) — Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Purity >95%.

SKU: BCACP-000851 Category:
Product SKUBCACP-000851
Product DescriptionRecombinant Human Superoxide dismutase [Cu-Zn] protein (SOD1) protein is expressed from E.Coli with Tag-Free. It contains 2-154aa. [Accession | P00441].
Uniprot No.P00441
Gene NamesSOD1
Purity>95% as determined by SDS-PAGE.
Expression SystemE.Coli
Expression Region2-154aa
SpeciesHomo sapiens (Human)
Tag InfoTag-Free
Molecular weight15.8 kDa
ActivityFully biologically active when compared to standard. The specific activity determined by fibrining lysis in agarose plate is 5.0x104 IU/mg.
BufferLyophilized from a 0.2 μm filtered 20 mM Tris-HCl, 0.5 M NaCl, 6% Trehalose, pH 8.0
StorageStore at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Alternative NamesALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Cu/Zn SOD; Cu/Zn superoxide dismutase; Epididymis secretory protein Li 44; HEL S 44; Homodimer; hSod1; Indophenoloxidase A; IPOA; Mn superoxide dismutase; SOD; SOD soluble; SOD1; SOD2; SODC; SODC_HUMAN; Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; Superoxide dismutase 1 soluble; Superoxide dismutase Cu Zn; Superoxide dismutase cystolic

Function

Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Catalyzes the oxidation of hydrogen sulfide (H2S) to sulfate, playing an important role in detoxifying H2S and limiting the accumulation of reactive sulfur species (RSS) such as persulfides and polysulfides.

Biological Context

Subcellular Location: Cytoplasm; Nucleus
Disease Association: Amyotrophic lateral sclerosis 1 (ALS1) : A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. 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 the cases. [The disease is caused by variants affecting the gene represented in this entry] | Spastic tetraplegia and axial hypotonia, progressive (STAHP) : An autosomal recessive, neurologic disorder characterized by loss of motor abilities in the first year of life, after which severe, progressive spastic tetraparesis develops. Affected individuals have severe axial hypotonia, hyperekplexia, hypertonia, and myokymia, reflecting upper motor neuron involvement. Cognitive development may be affected. [The disease is caused by variants affecting the gene represented in this entry]

Product Specifications

Recombinant Human Superoxide dismutase [Cu-Zn] protein (SOD1) (Active) is a recombinant protein from Homo sapiens (Human), expressed in E.Coli, covering amino acids 2-154aa, with Tag-Free tag, molecular weight 15.8 kDa, purity >95% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications.

Recommended applications depend on the target, product format, and product-specific validation data. Refer to the product datasheet or Certificate of Analysis (CoA), or contact BioCrest Sci for application guidance.

Biological Activity: Fully biologically active when compared to standard. The specific activity determined by fibrining lysis in agarose plate is 5.0x104 IU/mg.

Storage: Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shipping & Lead Time: Availability, shipping conditions, and estimated lead time are confirmed in the formal quotation or order confirmation based on the product format, destination, and order requirements.

Protein Biology

Function

Destroys radicals which are normally produced within the cells and which are toxic to biological systems (PubMed:18948262, PubMed:24140062). Catalyzes the oxidation of hydrogen sulfide (H2S) to sulfate, playing an important role in detoxifying H2S and limiting the accumulation of reactive sulfur species (RSS) such as persulfides and polysulfides (PubMed:36630448)

Subcellular Location

Cytoplasm; Nucleus

Disease Association

Amyotrophic lateral sclerosis 1 (ALS1) : A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. 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 the cases. [The disease is caused by variants affecting the gene represented in this entry] | Spastic tetraplegia and axial hypotonia, progressive (STAHP) : An autosomal recessive, neurologic disorder characterized by loss of motor abilities in the first year of life, after which severe, progressive spastic tetraparesis develops. Affected individuals have severe axial hypotonia, hyperekplexia, hypertonia, and myokymia, reflecting upper motor neuron involvement. Cognitive development may be affected. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

Homodimer; non-disulfide-linked (By similarity). Homodimerization may take place via the ditryptophan cross-link at Trp-33. Heterodimer with SOD1 (PubMed:31292775). The heterodimer CCS:SOD1 interacts with SLC31A1; this heterotrimer is Cu(1+)-mediated and its maintenance is regulated through SOD1 activation (PubMed:31292775). Interacts with DAOA; the interaction is direct (PubMed:30037290)

Gene: SOD1  |  Organism: Homo sapiens  |  Synonyms: Hydrogen sulfide oxidase; Superoxide dismutase 1
Key Publications

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Availability, dispatch timing, shipping conditions, and delivery estimates depend on the product format, destination, and order requirements. These details are confirmed in the formal quotation or order confirmation.

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Quality-control data such as identity, purity, endotoxin level, or biological activity are provided only when specified on this product page, the datasheet, or Certificate of Analysis (CoA). Biological activity is not implied unless explicitly stated.

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