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POMK Antibody

  • 货号：
    CSB-PA863985LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA863985LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA863985LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) POMK Polyclonal antibody
  • Uniprot No.：
    Q9H5K3
  • 基因名：
    POMK
  • 别名：
    FLJ23356 antibody; MDDGA12 antibody; POMK antibody; Probable inactive protein kinase-like protein SgK196 antibody; Protein kinase like protein SgK196 antibody; Protein kinase-like protein SgK196 antibody; Protein O-mannose kinase antibody; SG196_HUMAN antibody; SGK196 antibody; Sugen kinase 196 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Protein O-mannose kinase protein (44-350AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，POMK Antibody (CSB-PA863985LA01HU)，的标记方式是Non-conjugated。对于POMK Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA863985LB01HU POMK Antibody, HRP conjugated ELISA
    FITC CSB-PA863985LC01HU POMK Antibody, FITC conjugated
    Biotin CSB-PA863985LD01HU POMK Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • 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：
    Protein O-mannose kinase that specifically mediates phosphorylation at the 6-position of an O-mannose of the trisaccharide (N-acetylgalactosamine (GalNAc)-beta-1,3-N-acetylglucosamine (GlcNAc)-beta-1,4-mannose) to generate phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-1,3-N-acetylglucosamine-beta-1,4-(phosphate-6-)mannose). Phosphorylated O-mannosyl trisaccharide is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Only shows kinase activity when the GalNAc-beta-3-GlcNAc-beta-terminus is linked to the 4-position of O-mannose, suggesting that this disaccharide serves as the substrate recognition motif.
  • 基因功能参考文献：
    1. POMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentations. PMID: 24925318
    2. Homozygous truncating mutations in POMK lead to congenital muscular dystrophies with secondary merosin deficiency, hypomyelination and intellectual disability. PMID: 24556084
    3. These findings suggest how mutations in GTDC2, B3GALNT2, and SGK196 disrupt dystroglycan receptor function and lead to congenital muscular dystrophy. PMID: 23929950
  • 相关疾病：
    Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A12 (MDDGA12); Muscular dystrophy-dystroglycanopathy limb-girdle C12 (MDDGC12)
  • 亚细胞定位：
    Endoplasmic reticulum membrane; Single-pass type II membrane protein.
  • 蛋白家族：
    Protein kinase superfamily, Ser/Thr protein kinase family, STKL subfamily
  • 组织特异性：
    Highest expression is observed in brain, skeletal muscle, kidney and heart in fetal and adult tissues.
  • 数据库链接：

    HGNC: 26267

    OMIM: 615247

    KEGG: hsa:84197

    STRING: 9606.ENSP00000331258

    UniGene: Hs.491646