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

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Code CSB-AP000121HU
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Product Details

Purity >95% as determined by SDS-PAGE and HPLC.
Endotoxin Less than 1.0 EU/μg as determined by LAL method.
Activity Fully biologically active when compared to standard. The potency per mg was determined by Pyrogallic Acid method and was found to be more than 1.0x104 IU/mg.
Target Names SOD1
Uniprot No. P00441
Research Area Cancer
Alternative Names ALS; 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
Species Homo sapiens (Human)
Source E.Coli
Expression Region 2-154aa
Mol. Weight 20 kDa
Protein Length Full Length of Mature Protein
Tag Info N-terminal 6xHis-tagged
Form Lyophilized powder
Buffer Lyophilized from a 0.2 µm filtered PBS, pH 7.4
Reconstitution We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
and FAQs
Protein FAQs
Storage Condition Store at -20°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time Basically, we can dispatch the products out in 5-10 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA Please contact us to get it.

Target Data

Function Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
Gene References into Functions
  1. These results show that secosterol aldehydes are increased in plasma of symptomatic amyotrophic lateral sclerosis rats, overexpressing multiple copies (~8 copies) of G93A mutant human SOD1, and represent a class of aldehydes that can potentially modify SOD1 enhancing its propensity to aggregate. PMID: 30142602
  2. Study results in transgenic mice carrying human SOD1 gene and analysis of cerebrospinal fluid as well as the spinal cord homogenate amyotrophic lateral sclerosis (ALS) patients suggest that metal-deficiency in mutant SOD1 at its copper-binding site is one of the earliest pathological features in SOD1-ALS. PMID: 29551730
  3. A stable core of the SOD2 that unfolds last and refolds first, and directly observe several distinct misfolded states that branch off from the native folding pathways at specific points after the formation of the stable core. PMID: 29192167
  4. The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis. PMID: 28711596
  5. the introduction of SOD1(G93A) and TDP43(A315T), established Amyotrophic lateral sclerosis (ALS)-related mutations, changed the subcellular expression and localization of RNAs within the neurons, showing a spatial specificity to either the soma or the axon. Altogether, we provide here the first combined inclusive profile of mRNA and miRNA expression in two ALS models at the subcellular level. PMID: 28300211
  6. Shorter activated partial thromboplastin time and increased SOD levels might be useful hemostatic markers in patients with type 2 diabetes mellitus. PMID: 30143488
  7. In this study we demonstrate dynamic changes in the number of calretinin- (CR) and neuropeptide Y-expressing (NPY) interneurons in the motor cortex of the familial hSOD1(G93A) ALS mouse model, suggesting their potential involvement in motor neuron circuitry defects PMID: 28294153
  8. Our results suggest that SOD1 mutation is the most common cause of amyotrophic lateral sclerosis(ALS) in Chinese populations and that the mutation spectrum of ALS varies among different ethnic populations PMID: 28291249
  9. Weak significance was observed for a protective effect of the TT genotype of rs1041740 in the SOD1 gene relative to Type 1 Diabetes development (OR 0.318, 95% CI 0.092-0.959, p = 0.056). PMID: 29924645
  10. SOD1 is S-acetylated in spinal cord homogenates from ALS and non-ALS subjects. The degree of S-acylation is highest for SOD1-CCS heterodimers and lowest for SOD1 monomers. PMID: 28120938
  11. Study suggests that endoplasmic reticulum stress increases the susceptibility of SOD1WT to aggregate during aging, operating as a possible risk factor for developing amyotrophic lateral sclerosis. PMID: 30038021
  12. Metallation and oxidation of SOD1 stabilize the native, mature conformation and decrease the number of detected excited conformational states. PMID: 29483249
  13. results thus shed light on the role of local unfolding and conformational dynamics in aggregation of SOD1 PMID: 29369331
  14. Certain SOD1 mutants, viz. His80Arg and Asp83Gly, were recognized that were more damaging to the Zn binding loop than all other mutants, leading to a loss of Zn binding with altered coordination of the Zn ion. Furthermore, the conformational stability, compactness, and secondary structural alteration of the His80Arg and Asp83Gly mutants were monitored using distinct parameters. PMID: 28271284
  15. describe here two cases of apparently sporadic amyotrophic lateral sclerosis associated with mutations, respectively, in SOD1 and TARDP genes PMID: 27494151
  16. that global changes in DNA methylation might contribute to the ALS phenotype in carriers of not fully penetrant SOD1 mutations PMID: 28859526
  17. The present study indicating that although the Ins/Del polymorphism of SOD1 is associated with the SOD1 expression levels, this polymorphism is not associated with the risk of dependency to heroin. PMID: 29165112
  18. The mutant human SOD1-G93A protein induced axonal and myelin degeneration during the progression of Amyotrophic Lateral Sclerosis in a mouse model and participated in axon remyelination and regeneration in response to injury. PMID: 29742495
  19. SOD1 oligomer and not the mature form of aggregated fibril is critical for the neurotoxic effects in the model of amyotrophic lateral sclerosis. PMID: 29666246
  20. Data suggest that serum SOD1 levels are decreased in patients with controlled or uncontrolled acromegaly as compared to healthy subjects; in acromegaly, SOD1 levels are not associated with MnSOD/SOD2 polymorphisms. PMID: 29046499
  21. Ovariectomy resulted in earlier disease onset and attenuated the anti-inflammatory and anti-apoptotic actions of estrogen in hSOD1-G93A transgenic mice. PMID: 29394243
  22. a measure of hydrogen bond stability in conformational states was studied with elastic network analysis of 35 SOD1 mutants. PMID: 29431095
  23. The genetic mutations of SOD1 caused amyotrophic lateral sclerosis PMID: 29478603
  24. the study provides a better understanding over the profound effect of mutation on SOD1, both structurally and functionally, using computational approaches. PMID: 28899654
  25. SOD1 amino acid residues forming these pathogenic hydrogen bonds are found in zinc-binding and electrostatic loops as well as at zinc-binding sites and are in contact with SOD1 aggregates, which implies that these regions are sensitive to perturbations from pathogenic mutations. PMID: 28950184
  26. show a clear variation of the different SOD1 mutants to associate with mitochondrial-enriched fractions with a correlation between mutation severity and this association PMID: 28715630
  27. Study shows that in senile cataracts, SOD1 expression decreased significantly. Both H3 and H4 were deacetylated at -600 bp of the SOD1 promoter of cataract lenses, and hypoacetylated at -1500, -1200, and -900 bp. In hypoacetylated histones, the hypoacetylation pattern differed among the cataracts sub-types. Further functional data provide evidence that histone acetylation plays an essential role in the regulation of SOD1. PMID: 27703255
  28. Study shows that SOD1 forms fibrillar aggregates under quiescent conditions at near-physiological pH, ionic strength, and temperature over a time frame of weeks; and that intermolecular disulfide bonds are not required for the protein to form aggregates, even in the absence of fibril seeds. Scrambling of intramolecular disulfide bonds is not required for aggregation. Urea denaturation increases aggregation lag time. PMID: 28585802
  29. SOD1 G93A mutant from familial amyotrophic lateral sclerosis cases binds VDAC1 with high affinity. PMID: 27721436
  30. Like other neurodegenerative diseases, misfolding of a specific protein is central to ALS. SOD1, the major constituent of the protein deposits in some familial and sporadic forms of ALS, propagates its misfolded conformation like prions, providing a plausible molecular basis for the focality and spreading of muscle weakness in ALS PMID: 28096265
  31. Data show that cholecystectomy patients with enhanced levels of superoxide dismutase (SOD1) appeared to have significantly lower number of analgesic oxycodone doses during the first 24 h postoperatively (NAD24). PMID: 29848712
  32. the structural changes and the alteration in distance between Zn and its binding residues which cause the loss of Zn binding was studied in details to delivery the foresight on the impact of the mutation in SOD1. PMID: 27555441
  33. antioxidant activity of erythrocyte SOD is associated with dementia severity. PMID: 28965606
  34. Results provide evidence that ALS mutant SOD1 inhibits axonal transport of mitochondria by inducing PINK1/Parkin-dependent Miro1 degradation. PMID: 28973175
  35. Study reveals presence of glial cell proliferation in both motor (brainstem) and non-motor (hippocampus) CNS structures of hSOD1G93A ALS rats starting already at the presymptomatic stage of the disease. A specific timeline of glial response is demonstrated in the brainstem of these animals with the activation of astrocytes coming first and before disease onset, followed by activation of microglia in the symptomatic phase. PMID: 28576725
  36. SOD1 mutations were present in 20% of familial amyotrophic lateral sclerosis (ALS) patients and 1.9% of sporadic ALS patients, while FUS mutations were responsible for 13.3% of familial ALS cases, and TARDBP mutations were rare in either familial or sporadic ALS cases. PMID: 27604643
  37. Significant association between the SOD1 Ins/Del polymorphism and age of onset in bipolar disorder type 1. PMID: 28750571
  38. Data provide evidence that metal binding, in addition of being necessary for SOD1 enzymatic activity, is a key factor in the aggregation process of SOD1. In particular, both demetalation and aberrant metal binding have been shown to promote misfolding and aggregation in SOD1 suggesting a possible role of metal binding in SOD1 pathological aggregation. [review] PMID: 28850080
  39. SOD1 heterodimerization rate is influenced by mutation and is correlated with survival times in amyotrophic lateral sclerosis. PMID: 27054659
  40. early stage influenza A virus infection induces autophagic degradation of antioxidant enzyme SOD1, thereby contributing to increased ROS generation and viral infectivity in alveolar epithelial cells. PMID: 29548827
  41. Computational investigation of the human SOD1 mutant, Cys146Arg, that directs familial amyotrophic lateral sclerosis, has been reported. PMID: 28621357
  42. Suggest a complex role of SOD1 in different processes leading to complications of liver cirrhosis. rs1041740 might be associated with the development of ascites and possibly plays a role in spontaneous bacterial peritonitis once ascites has developed. PMID: 28403123
  43. SOD1 gene polymorphisms associated with susceptibility to noise-induced hearing loss. PMID: 29072670
  44. all affected members, except the proband's father, who was unavailable for DNA analysis, showed a heterozygous mutation (c.125G>A) in exon 2 of the SOD1 gene. We found that the executive domain, attention domain, language function, calculation tasks and memory were significantly impaired in the patients with ALS compared to the healthy family members. PMID: 26069299
  45. Slowly upper and lower motor neuron degeneration, even with non-motor clinical features, should prompt a sequencing of SOD1 PMID: 27892702
  46. research demonstrated that erysipelas infection predisposition and its clinical characteristics are affected by age, sex and SNPs found in SOD1, SOD2, and catalase genes; presence of SOD1 G7958 alleles was linked to erysipelas' predisposition; G and A alleles of SOD1 G7958A individually were associated with lower limbs and higher body part localizations of the infection, respectively PMID: 28512644
  47. Low SOD1 Expression Is Associated with Postoperative Pain. PMID: 29374733
  48. Our data indicate that SOD1 is directly or indirectly involved in ALS oligodendrocyte pathology and suggest that in this cell type, some damage might be irreversible. PMID: 27688759
  49. Gelsolin enhances the invasive capacity of colon cancer cells via elevating intracellular superoxide (O2.-) levels by interacting with Cu/ZnSOD, and gelsolin gene expression positively correlates with urokinase plasminogen activator (uPA), an important matrix-degrading protease invovled in cancer invasion. PMID: 27391159
  50. The observation that beta-strand 5 is among the first to unfold here, but the last to unfold in simulations of loop-truncated SOD1, could imply the existence of an evolutionary compensation mechanism, which would stabilize beta-strands flanking long loops against their entropic penalty by strengthening intramolecular interactions. PMID: 28629863
  51. The various biochemical and structure-based hypotheses proposed for mutant SOD1-associated Amyotrophic Lateral Sclerosis are discussed [REVIEW] PMID: 28132491
  52. Using different methods to detect misfolded SOD1, multiple misfolded SOD1 antibodies raised against different epitopes, appropriate parallel controls and by controlling for staining artefacts through the comparison of different antigen retrieval methods, study demonstrated that misfolded SOD1 is not a contributing component of sporadic amyotrophic lateral sclerosis. PMID: 28247063
  53. Its mutation is a genetic cause of amyotrophic lateral sclerosis. PMID: 28222900
  54. The results show that the docking of the electrostatic loop to the rest of SOD1 plays a role in amyotrophic lateral sclerosis (ALS) pathogenesis, in support of that structure acting as a solvent barrier for the metal site. The results provide a unified pathogenic mechanism for five different ALS-linked mutations of SOD1. PMID: 27603566
  55. SOD1 is the seeding particle responsible for the spread of SOD1-familial amyotrophic lateral sclerosis neurodegeneration from its initial onset site PMID: 28877271
  56. The results suggest that SFH prevents the degeneration of pancreatic islets via increasing SOD while hyperglycemia is alleviated by maintaining beta cell mass in type 2 diabetes model mice. PMID: 28919426
  57. study thus implies that the WT and mutant SOD1 indeed converge on a common mechanism for gain of cytotoxicity by abnormally interacting with membranes. Moreover, any genetic/environmental factors which can delay or impair its maturation might act to transform SOD1 into cytotoxic forms with the acquired capacity to abnormally interact with membranes. PMID: 27378311
  58. Data suggest that multiple non-native species of misfolded SOD1 may exist in the SOD1(G93A) rat model, some of which are associated with mitochondrial damage, highlighting that variable potency/toxicity of different SOD1 species is possible even when only one SOD1 mutation is present. PMID: 27121871
  59. SOD1 is one of the most commonly mutated genes in ALS. PMID: 27297615
  60. superoxide dismutase function of SOD1 might not be required to preserve DNA integrity in motor neurons, at least when the function of TDP-43 is unaltered PMID: 28832631
  61. The results suggest that the 50 bp deletion has a moderate reducing effect on SOD1 synthesis. PMID: 27002425
  62. deletion-rescue experiments show that a respiration-defective mutant of SOD1 is also impaired in its ability to rescue cells from toxicity caused by SOD1 deletion PMID: 28739857
  63. Factors released in vitro from astrocytes derived from SOD-1 transgenic mice cause spinal motoneuron death and consequent neuromuscular dysfunction in vivo. PMID: 28128448
  64. This study identified a new pathology hallmark in SOD1G93A ALS mice: a glutamatergic sensory neuron dendropathy restricted to olfactory bulb mitral cells and retinal ganglionic cells. PMID: 28859334
  65. demonstrated that enervating the SOD1 electrostatic loop can lead to an experimentally observed gain of interaction (GOI) responsible for the formation of SOD1 amyloid-like filaments PMID: 28472188
  66. Sod1 upregulation was noted in the R region of the nonaneurysmal type 1 L/R morphotype. Region-specific transcription profiles of Sod on the basis of BAV morphotype deepen our understanding of its associated aortopathy and provide biological insight on the asymmetric dilatation pattern. PMID: 28185644
  67. This study demonstrated that the injection into isolated Aplysia neurons of oligomeric forms of a mutant G85R SOD1 associated with ALS in both humans and transgenic mice reduces net outward K+ current and increases excitability. PMID: 28119399
  68. sodium channel currents in oocytes expressing either wild-type or mutant (A4V) SOD1 protein PMID: 27072680
  69. findings indicate that CuZn-SOD is able to response to the hypomagnetic field stress and suggest it a mediator of the hypomagnetic field effect. PMID: 28447293
  70. Data suggest that Ccs1 activates immature Sod1 by delivering copper and facilitating oxidation of intramolecular disulfide bond in Sod1; Ccs1 binding exposes an electropositive cavity and proposed "entry site" for copper ion delivery on the apoenzyme. (Ccs1 = copper chaperone for superoxide dismutase; Sod1 = copper-zinc superoxide dismutase) PMID: 28533431
  71. The cause of aggregation and reduced Zn binding affinity by G85R mutation in SOD1 rendering amyotrophic lateral sclerosis has been described. PMID: 28321933
  72. propose an alternative pathway of mutant SOD1 misfolding that is responsible for oligomerization in the pathologies of the disease. PMID: 27977888
  73. In cells that overexpress a genetic variant of SOD1, newly made mutant SOD1 was rapidly captured by pathologic intracellular inclusions. PMID: 27727458
  74. Findings show that a phosphomimetic mutation, T2D, thermodynamically stabilizes SOD1 even in the context of a strongly SOD1-destabilizing mutation, A4V, one of the most prevalent and aggressive amyotrophic lateral sclerosis -associated mutations. This stabilization protects against formation of toxic SOD oligomers and positively impacts motor neuron survival in cellular assays. PMID: 27667694
  75. Mutated obese carries of SOD1 -251, SOD2 47 and CAT -262 are associated with a higher distribution of fat in comparison with obese wild-type carriers. PMID: 27751366
  76. Data suggest two conformational-change scenarios, one local to the electrostatic loop (ESL) at the complex interface, and a second displacement at the ESL of the otherdimeric subunit. PMID: 27591900
  77. Steered molecular dynamics analysis was used to study the phenomenon of SOD1 proteopathy in ALS, suggests that the increased backbone mobility transforms certain surface regions of the misfolded SOD1 into structural equivalents of the interaction hot spots and has guided the identification of SOD1-derived peptides that bind SOD1 and alter the course of the amyloid aggregation of fALS SOD1 mutants. PMID: 27540759
  78. Results reveal that SOD1G93A sensory neurons accumulate mutant/misfolded Cu/Zn-superoxide dismutase but, surprisingly, do not suffer from endoplasmic reticulum stress and unfolded protein response activation PMID: 27241719
  79. e observed significant changes in expression of Sig1R, SOD1, SOD2 due to different oxygen concentrations. ANOVA analysis revealed significant interactions between studied parameters mainly in hypoxia conditions in SW480 cells and between Sig1R and SOD2 in SW620 cells PMID: 27829319
  80. Drosophila carrying homozygous mutations G85R, H48R, and H71Y exhibited neurodegeneration, locomotor deficits, and shortened life span. Drosophila knock-in model captures important aspects of human SOD1-based ALS. PMID: 27974499
  81. Haplotype analysis identified the haplotype with T-allele of rs1041740 and that with T-allele of rs17880487 of superoxide dismutase-1 as increasing and decreasing susceptibility for cardiovascular mortality, and it had complementary single nucleotide polymorphism sequences. PMID: 27755600
  82. By utilizing cumate inducible SOD1 cells, we also showed that knock-down or pharmacologic depletion of cIAPs leads to H2O2 induced cytotoxicity in mSOD1 expressing cells. Altogether, our results reveal a novel role of cIAPs in FALS-associated mutant SOD1 regulation. PMID: 27773815
  83. The potential protective effect without statistical relationships were also observed for other genotypes and alleles studied polymorphic variants of antioxidant enzymes in CD and CAT- 262C / T and + 35 A / C SOD1 in UC. Conducted our audit should be extended to more group of patients in order to assess whether or not to confirm the observed during analysis, the protective effect of CAT-262 C / T in ulcerative colitis and PMID: 28141554
  84. No significant differences in SOD and GPx activity both in plasma and saliva were found between Crohn's disease remission group and the control group. PMID: 28011951
  85. Gene expression profiling and pathway analysis followed by pharmacological screening identified activated ERK and JNK signaling as key drivers of neurodegeneration in mutant SOD1 motor neurons. PMID: 28366453
  86. Superoxide dismutase and catalase activities were lower in type 1 myotonic dystrophy patients compared to healthy controls PMID: 26817806
  87. These results strongly suggest that the thioredoxin and glutaredoxin systems are the key regulators for hSOD1 aggregation and may play critical roles in the pathogenesis of Amyotrophic Lateral Sclerosis. PMID: 27261461
  88. The ROS levels of the study group decreased obviously before irradiation (P<0.01), however, the radiation-induced ROS of the study group was at a high level even when irradiation had been terminated for 2 h (P<0.01). Moreover, NOX2 and NOX4 levels and total SOD activity decreased (P<0.01), while the levels of SOD1 were stably maintained (P>0.05). PMID: 28260074
  89. SOD1 has a role in amyotrophic lateral sclerosis disease phenotype PMID: 26878886
  90. Data show that antioxidant enzymes SOD2 and GPX1 expression and GPX1 and SOD1 activity were significantly higher in patients at diagnosis of bladder cancer (BC) in comparison to controls. PMID: 28179340
  91. Study showed that endothelial cells from human umbilical vein have a reduced activity and gene expression of the "classic" antioxidant enzymes (Cu,Zn-superoxide dismutase, catalase, and Se-containing glutathione peroxidase). At the same time, a high expression level of peroxiredoxin genes was identified in the same endothelial cells PMID: 28058676
  92. a structural model for protofibril capping of SOD1 filaments by alphaB-crystallin PMID: 27496206
  93. The effects of oleuropein (OL) on hydrogen peroxide-induced oxidative stress in L02 cells showed that SOD1, GPx1, and catalase levels were all increased, and suggested that OL is a potent antioxidant and may be therapeutically useful in liver disease prevention. PMID: 27914828
  94. The toxicity of the misfolded wild-type SOD1 is involved in the pathogenesis of sporadic Amyotrophic lateral sclerosis. [review] PMID: 26563614
  95. rs4998557 polymorphism associated with susceptibility to sudden sensorineural hearing loss PMID: 26882452
  96. The purpose of the study was to evaluate the plasma level of superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GPX) and the association between polymorphic variants in genes encoding for GPx1, SOD, CAT and the risk of distal symmetric polyneuropathy in type 2 diabetes mellitus patients. PMID: 26674569
  97. This study identified the L84F mutation in SOD1 in a amyotrophic lateral sclerosis family from North India PMID: 26630559
  98. Study found expression of several mutated forms of SOD1 in the NSC-34 motor neuronal cells induces the formation of cytosolic and sometimes nuclear aggregates containing the SUMO-1 protein and showed that the formation of these aggregates can be modulated by action on the K75 SUMOylation site PMID: 26605782
  99. the effects of oxidative modification on SOD1 monomer and homodimer stability PMID: 27074676
  100. The expression of hSOD1 in the liver of Sod1(-/-) mice significantly improved the lifespan of Sod1(-/-) mice; however, the lifespan of the Sod1(-/-)/hSOD1(alb) mice was still significantly shorter than wild type mice. PMID: 26839948

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Involvement in disease Amyotrophic lateral sclerosis 1 (ALS1)
Subcellular Location Cytoplasm, Mitochondrion, Nucleus
Protein Families Cu-Zn superoxide dismutase family
Database Links

HGNC: 11179

OMIM: 105400

KEGG: hsa:6647

STRING: 9606.ENSP00000270142

UniGene: Hs.443914

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