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OPA3 Antibody

  • 中文名稱：
    OPA3兔多克隆抗體
  • 貨號(hào)：
    CSB-PA880985ESR1HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA880985ESR1HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human gastric cancer using CSB-PA880985ESR1HU at dilution of 1:100
    • Immunofluorescent analysis of Hela cells using CSB-PA880985ESR1HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) OPA3 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    OPA3
  • 別名：
    OPA3 antibody; Optic atrophy 3 protein antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Optic atrophy 3 protein (25-179AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
    IF 1:50-1:200
  • Protocols：
  • 儲(chǔ)存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    May play some role in mitochondrial processes.
  • 基因功能參考文獻(xiàn)：
    1. Mutations of the OPA3 gene can cause either autosomal dominant or autosomal recessive optic atrophy. PMID: 25205859
    2. Report the results of a comprehensive study on OPA3 mutations, including the mutation spectrum and its prevalence in a large cohort of ADOA patients, the associated clinical phenotype and the functional characterisation of a newly identified OPA3 mutant. PMID: 24136862
    3. A novel missense mutation identifies OPA3 as the cause of a complex neurological disorder with marked lower limb dystonia. PMID: 23700088
    4. OPA1 mutations are the most common genetic defects identified in patients with suspected Autosomal-dominant optic atrophy (DOA), whereas OPA3 mutations are very rare in isolated optic atrophy cases. PMID: 21036400
    5. OPA3, as an integral mitochondrial outer membane perotein, has a crucial role in mitochondrial fission, and provides a direct link between mitochondrial morphology and optic atrophy. PMID: 20372962
    6. findings thus place the cellular metabolic defect of 3-methylglutaconic aciduria type III in the mitochondrion rather than the peroxisome and implicate loss of OPA3A rather than gain of OPA3B in disease etiology. PMID: 20350831
    7. two missense mutations in OPA3 in two families affected by autosomal dominant optic atrophy and cataract (ADOAC) PMID: 15342707
    8. patient with Costeff syndrome presenting the characteristics of this syndrome and the single-nucleotide polymorphic gene that causes this disease. PMID: 16838891
    9. OPA1 mutations, mtDNA mutations, and OPA3 mutations in 980 patients Leber's hereditary optic neuropathy and autosomal dominant optic atrophy PMID: 19319978
    10. The mouse ortholog of OPA3 purifies with mitochondrial inner membranes. PMID: 12865426
    11. The mouse ortholog of OPA3 purifies with mitochondria PMID: 18614015
    12. type III 3-methylglutaconic aciduria (optic atrophy plus syndrome, or Costeff optic atrophy syndrome): identification of the OPA3 gene and its founder mutation in Iraqi Jews PMID: 11668429

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  • 相關(guān)疾病：
    3-methylglutaconic aciduria 3 (MGA3); Optic atrophy 3 (OPA3)
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    OPA3 family
  • 組織特異性：
    Ubiquitous. Most prominent expression in skeletal muscle and kidney.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 8142

    OMIM: 165300

    KEGG: hsa:80207

    UniGene: Hs.466945