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PPA2 Antibody

  • 货号：
    CSB-PA018411GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9H2U2
  • 基因名：
  • 别名：
    HSPC124 antibody; Inorganic pyrophosphatase 2 antibody; Inorganic pyrophosphatase 2, mitochondrial antibody; IPYR2_HUMAN antibody; mitochondrial antibody; Ppa2 antibody; PPase 2 antibody; PPase2 antibody; Pyrophosphatase (inorganic) 2 antibody; Pyrophosphatase SID6 306 antibody; Pyrophosphatase SID6-306 antibody; Pyrophosphate phospho-hydrolase 2 antibody; SID6 306 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PPA2
  • 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 0.02% 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
  • 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：
    Hydrolyzes inorganic pyrophosphate. This activity is essential for correct regulation of mitochondrial membrane potential, and mitochondrial organization and function.
  • 基因功能参考文献：
    1. findings confirm the pathogenicity of PPA2 mutations and suggest that PPA2 is a cardiomyopathy-associated protein, which has a greater physiological importance in mitochondrial function than previously recognized PMID: 27523597
    2. data demonstrate that PPA2 is an essential gene in yeast and that biallelic mutations in PPA2 cause a mitochondrial disease leading to sudden cardiac arrest in infants PMID: 27523598
    3. Single nucleotide polymorphisms in PPA2 is associated with response to antipsychotic agents in schizophrenia. PMID: 23241943
    4. We think that PP2A can be one of the key components to regulate the fusion of various endocytotic compartments and /or the trafficking along the microtubules. PMID: 19067239
    5. No pathogenic mutations were identified in the PPA2 gene in patients with mitochondrial DNA depletion syndromes (MDS). PMID: 16300924

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  • 相关疾病：
    Sudden cardiac failure, alcohol-induced (SCFAI); Sudden cardiac failure, infantile (SCFI)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    PPase family
  • 组织特异性：
    Detected in brain, gastric carcinoma, lung, ovary, skeletal muscle, umbilical cord blood and a cell line derived from kidney proximal tubule epithelium.
  • 数据库链接：

    HGNC: 28883

    OMIM: 609988

    KEGG: hsa:27068

    STRING: 9606.ENSP00000343885

    UniGene: Hs.654957