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GCSH Antibody

  • 中文名稱:
    GCSH兔多克隆抗體
  • 貨號:
    CSB-PA009335GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • 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.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IHC,IF
  • Protocols:
  • 儲存條件:
    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.

產品評價

靶點詳情

  • 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:
    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
  • 相關疾病:
    Non-ketotic hyperglycinemia (NKH)
  • 亞細胞定位:
    Mitochondrion.
  • 蛋白家族:
    GcvH family
  • 數據庫鏈接:

    HGNC: 4208

    OMIM: 238330

    KEGG: hsa:2653

    STRING: 9606.ENSP00000319531

    UniGene: Hs.546256



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