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    Sulfite oxidase Rabbit pAb (bs-10426R)  
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    產(chǎn)品編號 bs-10426R
    英文名稱(chēng) Sulfite oxidase Rabbit pAb
    中文名稱(chēng) 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.  
    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
    克 隆 號
    交叉反應 Rat (predicted: Human,Mouse,Dog,Horse)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 52 kDa
    檢測分子量
    細胞定位 細胞漿 細胞膜 線(xiàn)粒體
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Sulfite oxidase: 351-450/545 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    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 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.

    SWISS:
    P51687

    Gene ID:
    6821

    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



    產(chǎn)品圖片
    Tissue/cell: rat kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-Sulfite oxidase Polyclonal Antibody, Unconjugated(bs-10426R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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