超氧化物歧化酶1抗体体elisa实验

英文名称  Anti-SOD1/SOD 

中文名称  超氧化物歧化酶1抗体(铜/锌过氧化物歧化酶SOD) 

超氧化物歧化酶1抗体(铜/锌过氧化物歧化酶SOD)别    名  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; SOD1; SOD2; SODC; Soluble indophenoloxidase A; Superoxide dismutase 1; Superoxide dismutase 1 soluble; Superoxide dismutase Cu Zn; Superoxide dismutase cystolic; SODC_HUMAN; Superoxide dismutase [Cu-Zn]; hSod1; Ipo1; SODC; Ipo-1; Sod-1; CuZnSOD; Cu/Zn-SOD; MGC107553; B430204E11Rik; superoxide-dimutase-1.  

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg 

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Cow   

产品类型  一抗    

研究领域  神经生物学 细胞凋亡 激酶和磷酸酶  

蛋白分子量  predicted molecular weight: 17kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human SOD1 (101-154aa) 

亚    型  IgG 

超氧化物歧化酶1抗体(铜/锌过氧化物歧化酶SOD)纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 SOD1 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. Mutations in the copper/ zinc superoxide dismutase (SOD1) gene are associated with 15- 20% of the familial forms of motoneuron disease. This 153 amino acid metalloenzyme is expressed in virtually all cells of all organisms above bacteria and is highly conserved across species, although some minor variations do occur. The incorporation of the mutated form of the SOD1 human gene into a transgenic mouse leads to the onset of the disease that closely resembles the human condition. These animals become weak at about 2-4 months of age and rapidly lose function, which results in death 4- 6 weeks later.

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.

Database links : UniProtKB/Swiss-Prot: P00441.2 

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。