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    Rabbit Anti-Sulfite oxidase/RBITC Conjugated antibody (bs-10426R-RBITC)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-10426R-RBITC
    英文名稱(chēng) Rabbit Anti-Sulfite oxidase/RBITC Conjugated antibody
    中文名稱(chēng) 羅丹明(RBITC)標記的亞硫酸鹽氧化酶抗體
    別    名 SO; EC 1.8.3.1; mitochondrial; Sulfite oxidase; Sulfite oxidase mitochondrial; Sulfite oxidase, mitochondrial precursor; Suox; SUOX_HUMAN.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 腫瘤  細胞生物  神經(jīng)生物學(xué)  信號轉導  轉錄調節因子  新陳代謝  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應
    產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 52kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Sulfite oxidase
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 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:
    Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008]

    Subunit:
    Homodimer.

    Subcellular Location:
    Mitochondrion intermembrane space.

    DISEASE:
    Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD) [MIM:272300]; also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.

    Similarity:
    Contains 1 cytochrome b5 heme-binding domain.

    Database links:

    Entrez Gene: 6821 Human

    Entrez Gene: 211389 Mouse

    Omim: 606887 Human

    SwissProt: P51687 Human

    SwissProt: Q8R086 Mouse

    Unigene: 558403 Human

    Unigene: 23352 Mouse



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