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SOD1 Recombinant Rabbit mAb (bsm-60838R)  
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產(chǎn)品編號 bsm-60838R
英文名稱 SOD1 Recombinant Rabbit mAb
中文名稱 超氧化物歧化酶1(銅/鋅過氧化物歧化酶SOD)重組兔單抗
別    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; 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.  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 10B4
交叉反應(yīng) Human,Mouse,Rat
產(chǎn)品應(yīng)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.
理論分子量 17 kDa
檢測分子量
細(xì)胞定位 細(xì)胞核 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SOD1 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 PBS, Glycerol, BSA.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



超氧化物歧化酶又稱銅/鋅過氧化物歧化酶SOD(Superoxide dismutase,簡稱SOD)是參與機體抗氧化(ROS,反應(yīng)性氧離子reactive oxygen species)防御機制和抵御細(xì)胞氧化損傷最重要的酶類之一, 廣泛存在于需氧生物、耐氧生物及某些厭氧微生物中,目前已知的SOD 主要分為三類,即胞質(zhì)中Cu/Zn-SOD(即SOD1)、線粒體中的Mn-SOD(即SOD2)和ec-SOD(即SOD3)。
超氧化物歧化酶-1SOD1的水平與很多生理反應(yīng)有關(guān),如:應(yīng)急,熱休克,紫外和X線照射等。SOD1水平降低能觸發(fā)AP2轉(zhuǎn)錄因子的激活。SOD1在臨床上對很多疾病診斷有重要意義。
產(chǎn)品圖片
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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