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PPA2 Antibody, FITC conjugated

  • 中文名稱(chēng):
    PPA2兔多克隆抗體, FITC偶聯(lián)
  • 貨號(hào):
    CSB-PA867137LC01HU
  • 規(guī)格:
    ¥880
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng):
    Rabbit anti-Homo sapiens (Human) PPA2 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 別名:
    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
  • 反應(yīng)種屬:
    Human
  • 免疫原:
    Recombinant Human Inorganic pyrophosphatase 2, mitochondrial protein (180-298AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    FITC
  • 克隆類(lèi)型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式:
    Liquid
  • 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:
    Hydrolyzes inorganic pyrophosphate. This activity is essential for correct regulation of mitochondrial membrane potential, and mitochondrial organization and function.
  • 基因功能參考文獻(xiàn):
    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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  • 相關(guān)疾病:
    Sudden cardiac failure, alcohol-induced (SCFAI); Sudden cardiac failure, infantile (SCFI)
  • 亞細(xì)胞定位:
    Mitochondrion.
  • 蛋白家族:
    PPase family
  • 組織特異性:
    Detected in brain, gastric carcinoma, lung, ovary, skeletal muscle, umbilical cord blood and a cell line derived from kidney proximal tubule epithelium.
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 28883

    OMIM: 609988

    KEGG: hsa:27068

    STRING: 9606.ENSP00000343885

    UniGene: Hs.654957



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