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SOD1 Recombinant Rabbit mAb (bsm-60838R)  
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25ul/800.00元
50ul/1400.00元
100ul/2500.00元
大包装/询价

产品编号 bsm-60838R
英文名称 SOD1 Recombinant Rabbit mAb
中文名称 超氧化物歧化酶1(铜/锌过氧化物歧化酶SOD)重组兔单抗
别    名 Superoxide Dismutase 1; ALS 1; ALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Amyotrophic lateral sclerosis 1; Cu/Zn SOD; Cu/Zn superoxide dismutase; Homodimer; Indophenoloxidase A; IPOA; Mn superoxide dismutase; SOD 1; SOD; SOD soluble; SOD2; SODC; So  
抗体来源 Rabbit
克隆类型 Recombinant
克 隆 号 10B4
交叉反应 Human,Mouse,Rat
产品应用 WB=1:500-1000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:50-100
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 17 kDa
检测分子量
细胞定位 细胞核 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SOD1 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 PBS, Glycerol, BSA.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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. [provided by RefSeq, Jul 2008]

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

Subunit:
Homodimer; non-disulfide linked. Homodimerization may take place via the ditryptophan cross-link at Trp-33. The pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not. The pathogenic variants ALS1 Arg-86 and Ala-94 interact with MARCH5, whereas wild-type protein does not.

Subcellular Location:
Cytoplasm. Note=The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.

Post-translational modifications:
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.

DISEASE:
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:
Belongs to the Cu-Zn superoxide dismutase family.

SWISS:
P00441

Gene ID:
6647

Database links:

Entrez Gene: 6647 Human

Entrez Gene: 20655 Mouse

Entrez Gene: 24786 Rat

Omim: 147450 Human

SwissProt: P00441 Human

SwissProt: P08228 Mouse

SwissProt: P07632 Rat

Unigene: 443914 Human

Unigene: 276325 Mouse

Unigene: 466779 Mouse

Unigene: 6059 Rat



超氧化物歧化酶又称铜/锌过氧化物歧化酶SOD(Superoxide dismutase,简称SOD)是参与机体抗氧化(ROS,反应性氧离子reactive oxygen species)防御机制和抵御细胞氧化损伤最重要的酶类之一, 广泛存在于需氧生物、耐氧生物及某些厌氧微生物中,目前已知的SOD 主要分为三类,即胞质中Cu/Zn-SOD(即SOD1)、线粒体中的Mn-SOD(即SOD2)和ec-SOD(即SOD3)。
超氧化物歧化酶-1SOD1的水平与很多生理反应有关,如:应急,热休克,紫外和X线照射等。SOD1水平降低能触发AP2转录因子的激活。SOD1在临床上对很多疾病诊断有重要意义。
产品图片
Sample: Lane 1: Mouse Cerebrum tissue lysates Lane 2: Mouse Liver tissue lysates Lane 3: Mouse Placenta tissue lysates Lane 4: Rat Cerebrum tissue lysates Lane 5: Rat Placenta tissue lysates Lane 6: Human HepG2 cell lysates Lane 7: Human HeLa cell lysates Lane 8: Human Jurkat cell lysates Lane 9: Human MCF-7 cell lysates Primary: Anti-SOD1 (bsm-60838R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 17 kDa Observed band size: 15 kDa
Paraformaldehyde-fixed, paraffin embedded (human colon); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (SOD1) Monoclonal Antibody, Unconjugated (bsm-60838R) at 1:300 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023)instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (SOD1) Monoclonal Antibody, Unconjugated (bsm-60838R) at 1:300 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023)instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (SOD1) Monoclonal Antibody, Unconjugated (bsm-60838R) at 1:300 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023)instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse colon); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (SOD1) Monoclonal Antibody, Unconjugated (bsm-60838R) at 1:300 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023)instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (SOD1) Monoclonal Antibody, Unconjugated (bsm-60838R) at 1:300 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023)instructionsand DAB staining.
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