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GCSH Antibody

  • 中文名稱：
    GCSH兔多克隆抗體
  • 貨號(hào)：
    CSB-PA009335GA01HU
  • 規(guī)格：
    ￥3,900
  • 其他：

產(chǎn)品詳情

  • Uniprot No.：
  • 基因名：
    GCSH
  • 別名：
    GCSHGlycine cleavage system H protein antibody; mitochondrial antibody; Lipoic acid-containing protein antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human GCSH
  • 免疫原種屬：
    Homo sapiens (Human)
  • 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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA,WB,IHC,IF
  • 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：
    The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST).
  • 基因功能參考文獻(xiàn)：
    1. Data indicate no mutation was found in glycine cleavage system protein-H (GCSH) and suggest that mutations in both glycine decarboxylase (GLDC) and aminomethyltransferase (AMT) are the main cause of glycine encephalopathy in Malaysian population. PMID: 25231368
    2. There is no detectable glycine cleavage enzyme activity in human skin fibroblasts. PMID: 21539457
    3. Heterozygous GCSH gene mutation in transient neonatal hyperglycinemia. PMID: 12402263
    4. Genetic analysis showed a non-previously described mutation affecting a consensus splice site (IVS2-1G > C 3) in the AMT gene encoding the T protein of the glycine cleavage system. PMID: 19299230
  • 相關(guān)疾?。?/div>
    Non-ketotic hyperglycinemia (NKH)
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    GcvH family
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 4208

    OMIM: 238330

    KEGG: hsa:2653

    STRING: 9606.ENSP00000319531

    UniGene: Hs.546256