Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. GCSH Antibody

GCSH Antibody

  • 货号：
    CSB-PA009335GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P23434
  • 基因名：
    GCSH
  • 别名：
    GCSHGlycine cleavage system H protein antibody; mitochondrial antibody; Lipoic acid-containing protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human GCSH
  • 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,IF
  • 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：
    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).
  • 基因功能参考文献：
    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
  • 相关疾病：
    Non-ketotic hyperglycinemia (NKH)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    GcvH family
  • 数据库链接：

    HGNC: 4208

    OMIM: 238330

    KEGG: hsa:2653

    STRING: 9606.ENSP00000319531

    UniGene: Hs.546256