超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体

英文名称    SOD1    

中文名称    超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体    

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; 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.    

超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体        

说 明 书    0.1ml  0.2ml      

研究领域    神经生物学  细胞凋亡  激酶和磷酸酶      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Cow,     

产品应用    IHC-P=1:100-500 IHC-F=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.    

分 子 量    17kDa    

细胞定位    细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

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

亚    型    IgG    

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    

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.    

超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体 产品介绍    background:
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.

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

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

超氧化物歧化酶1/铜,锌过氧化物歧化酶SOD抗体 

hz-0293M-Gold Mouse Anti-rat IgG/Gold  胶体金标记的小鼠抗大鼠IgG

hz-0293M-AP Mouse Anti-rat IgG/AP  碱性磷酸酶（AP）标记的小鼠抗大鼠IgG

hz-0293M-FIThz Mouse Anti-rat IgG/FITC  FITC标记的小鼠抗大鼠IgG

hz-0293M-HRP Mouse Anti-rat IgG/HRP  辣根过氧化物酶标记的小鼠抗大鼠IgG

hz-0293M-RBIThz Mouse Anti-rat IgG/RBITC  罗丹明标记的小鼠抗大鼠IgG

hz-0293M-Bio Mouse Anti-rat IgG/Bio  生物素标记的小鼠抗大鼠IgG

hz-0293M-PE-hzy7 Mouse Anti-rat IgG/PE-Cy7  PE-Cy7标记的小鼠抗大鼠IgG

hz-0326M-Bio Mouse Anti-Bov IgG/Bio  生物素标记的小鼠抗牛IgG

hz-0326M-hzy3 Mouse Anti-Bov IgG/Cy3  Cy3标记的小鼠抗牛IgG

hz-0326M-AP Mouse Anti-Bov IgG/AP  碱性磷酸酶（AP）标记的小鼠抗牛IgG

hz-0326M-PE-hzy7 Mouse Anti-Bov IgG/PE-Cy7  PE-Cy7标记的小鼠抗牛IgG

hz-0326M-Gold Mouse Anti-Bov IgG/Gold  胶体金标记的小鼠抗牛IgG

hz-0326M-HRP Mouse Anti-Bov IgG/HRP  辣根过氧化物酶标记的小鼠抗牛IgG

hz-0326M-RBIThz Mouse Anti-Bov IgG/RBITC  罗丹明标记的小鼠抗牛IgG

hz-0326M-FIThz Mouse Anti-Bov IgG/FITC  FITC标记的小鼠抗牛IgG

hz-0326M-hzy5 Mouse Anti-Bov IgG/Cy5  Cy5标记的小鼠抗牛IgG

hz-0326M-hzy5.5 Mouse Anti-Bov IgG/Cy5.5  Cy5.5标记的小鼠抗牛IgG

hz-0326M-hzy7 Mouse Anti-Bov IgG/Cy7  Cy7标记的小鼠抗牛IgG

hz-0326M-PE Mouse Anti-Bov IgG/PE  PE标记的小鼠抗牛IgG

hz-0326M-PE-hzy3 Mouse Anti-Bov IgG/PE-Cy3  PE-Cy3标记的小鼠抗牛IgG

hz-0326M-PE-hzY5 Mouse Anti-Bov IgG/PE-CY5  PE-CY5标记的小鼠抗牛IgG

hz-0326M-APhz Mouse Anti-Bov IgG/APC  APC标记的小鼠抗牛IgG

hz-0326M-AF350 Mouse Anti-Bov IgG/Alexa Fluor 350  Alexa Fluor 350标记的小鼠抗牛IgG

hz-0326M-AF488 Mouse Anti-Bov IgG/Alexa Fluor 488  Alexa Fluor 488标记的小鼠抗牛IgG

hz-0326M-AF555 Mouse Anti-Bov IgG/Alexa Fluor 555  Alexa Fluor 555标记的小鼠抗牛IgG

hz-0326M-AF647 Mouse Anti-Bov IgG/Alexa Fluor 647  Alexa Fluor 647标记的小鼠抗牛IgG

hz-0326M-PE-hzy5.5 Mouse Anti-Bov IgG/PE-Cy5.5  PE-Cy5.5标记的小鼠抗牛IgG

hz-0358M-hzy3 Mouse Anti-Guinea pig IgG/Cy3  Cy3标记的小鼠抗豚鼠IgG

hz-0358M-hzy5 Mouse Anti-Guinea pig IgG/Cy5  Cy5标记的小鼠抗豚鼠IgG

hz-0358M-hzy5.5 Mouse Anti-Guinea pig IgG/Cy5.5  Cy5.5标记的小鼠抗豚鼠IgG

hz-0358M-hzy7 Mouse Anti-Guinea pig IgG/Cy7  Cy7标记的小鼠抗豚鼠IgG

hz-0358M-PE Mouse Anti-Guinea pig IgG/PE  PE标记的小鼠抗豚鼠IgG