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OPA3 Antibody

  • 货号：
    CSB-PA016342GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9H6K4
  • 基因名：
    OPA3
  • 别名：
    OPA3 antibody; Optic atrophy 3 protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human OPA3
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    May play some role in mitochondrial processes.
  • 基因功能参考文献：
    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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  • 相关疾病：
    3-methylglutaconic aciduria 3 (MGA3); Optic atrophy 3 (OPA3)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    OPA3 family
  • 组织特异性：
    Ubiquitous. Most prominent expression in skeletal muscle and kidney.
  • 数据库链接：

    HGNC: 8142

    OMIM: 165300

    KEGG: hsa:80207

    UniGene: Hs.466945