| 產(chǎn)品編號 | bs-1079R-BF647 |
| 英文名稱1 | Rabbit Anti-SOD1/BF647 Conjugated antibody |
| 中文名稱 | BF647標(biāo)記的超氧化物歧化酶1/銅,鋅過氧化物歧化酶SOD抗體 |
| 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; 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. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 神經(jīng)生物學(xué) 細(xì)胞凋亡 激酶和磷酸酶 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, Mouse, (predicted: Rat, Cow, ) |
| 產(chǎn)品應(yīng)用 |
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 17kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SOD1 (101-154aa) |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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. Database links: Entrez Gene: 6647 Human Entrez Gene: 20655 Mouse Omim: 147450 Human SwissProt: P00441 Human SwissProt: P08228 Mouse 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. 超氧化物歧化酶又稱銅/鋅過氧化物歧化酶SOD(Superoxide dismutase，簡稱SOD)是參與機(jī)體抗氧化（ROS，反應(yīng)性氧離子reactive oxygen species）防御機(jī)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。 |
