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TCAP Antibody

  • 货号：
    CSB-PA023264ESR1HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA023264ESR1HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA023264ESR1HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TCAP Polyclonal antibody
  • Uniprot No.：
    O15273
  • 基因名：
    TCAP
  • 别名：
    19 kDa sarcomeric protein antibody; CMD1N antibody; CMH25 antibody; LGMD2G antibody; Limb girdle muscular dystrophy 2G (autosomal recessive) antibody; T-cap antibody; Tcap antibody; TELE antibody; Telethonin antibody; TELT_HUMAN antibody; Titin cap protein antibody; Titin-cap antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Telethonin protein (1-167AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 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 0.02% sodium azide, 50% glycerol, pH7.3.
  • 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：
    Muscle assembly regulating factor. Mediates the antiparallel assembly of titin (TTN) molecules at the sarcomeric Z-disk.
  • 基因功能参考文献：
    1. based on the crystal structure in which Z1Z2 and telethonin1-90 assemble into a 2:1 complex, a single chain fusion protein was designed, comprising two Z1Z2 modules that are connected by flexible linkers N- and C-terminally of the telethonin1-90. Expression of this fusion protein, named ZTZ, affords high yields of soluble expressed and purified protein PMID: 28811266
    2. Data indicate LGMD2G protein TCAP association with limb girdle muscular dystrophy 2G (LGMD2G). PMID: 25055047
    3. Report TCAP mutations in dilated cardiomyopathy. PMID: 24037902
    4. cardiac telethonin is constitutively bis-phosphorylated and suggest that such phosphorylation is critical for normal telethonin function, which may include maintenance of transverse tubule organization and intracellular Ca(2+) transients. PMID: 24280220
    5. This study identified no pathogenic mutations in BAG3, MATR3, PTRF or TCAP in Australian muscular dystrophy. PMID: 21683594
    6. reduced expression of dystrophin and titin is associated with the pathophysiology of dilated cardiomyopathy, and TNF-alpha may modulate the expression of these proteins via NF-kappaB pathway. PMID: 20373002
    7. Genetic studies of tcap in zebrafish suggested that pathogenesis in LGMD2G is due to a disruption of sarcomere-T-tubular interaction, but not of sarcomere assembly per se. PMID: 19679566
    8. review of telethonin and other new proteins of the Z-disc of skeletal muscle PMID: 11699871
    9. telethonin protein levels seems to be at least in part regulated by neuronal activity and is thus linked to the dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle. PMID: 11763198
    10. telethonin may play a role in linking titin filaments at the Z-disk periphery PMID: 12446666
    11. TCAP mutations identified which are associated with hypertrophic cardiomyopathy. PMID: 15582318
    12. The titin Z1Z2-telethonin complex resist considerable mechanical force through beta strand crosslinking, suggesting that telethonin is an important component of the N-terminal titin anchor. PMID: 16531234
    13. A dimer of two titin/telethonin complexes is formed within the crystal environment, potentially indicating the formation of higher oligomers. PMID: 16713295
    14. Hypertension induced expression of prohypertrophic BMP10, and the hypertrophic effect of BMP10 was modulated, at least in part, by its binding to Tcap at the Z disc. PMID: 17921333
    15. telethonin is a sodium channel-interacting protein, and its mutations can alter Na(v)1.5 kinetics and may play a role in intestinal pseudo-obstruction PMID: 18408010
    16. Telethonin might be involved in CVB3-mediated cell damage and in the resulting cardiac dysfunction due to the interaction with Siva. PMID: 18849585
    17. Oncogenomic recombination hotspot around the PPP1R1B-STARD3-TCAP-PNMT-PERLD1-ERBB2-C17orf37-GRB7 amplicon at human chromosome 17q12 is closely linked to evolutionary recombination hotspot around the GSDML-GSDM locus. PMID: 15010812

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  • 相关疾病：
    Cardiomyopathy, familial hypertrophic 25 (CMH25); Limb-girdle muscular dystrophy 2G (LGMD2G)
  • 亚细胞定位：
    Cytoplasm, myofibril, sarcomere.
  • 组织特异性：
    Heart and skeletal muscle.
  • 数据库链接：

    HGNC: 11610

    OMIM: 601954

    KEGG: hsa:8557

    STRING: 9606.ENSP00000312624

    UniGene: Hs.77628