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  4. Recombinant Human Telethonin (TCAP)

Recombinant Human Telethonin (TCAP)

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  • 货号：
    CSB-EP023264HU
  • 规格：
    ￥1836
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 90% as determined by SDS-PAGE.
  • 基因名：
    TCAP
  • Uniprot No.：
  • 别名：
    19 kDa sarcomeric protein; CMD1N; CMH25; LGMD2G; Limb girdle muscular dystrophy 2G (autosomal recessive); T-cap; Tcap; TELE; Telethonin; TELT_HUMAN; Titin cap protein; Titin-cap
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length
  • 来源：
    E.coli
  • 分子量：
    46.1kDa
  • 表达区域：
    1-167aa
  • 氨基酸序列
    MATSELSCEVSEENCERREAFWAEWKDLTLSTRPEEGCSLHEEDTQRHETYHQQGQCQVLVQRSPWLMMRMGILGRGLQEYQLPYQRVLPLPIFTPAKMGATKEEREDTPIQLQELLALETALGGQCVDRQEVAEITKQLPPVVPVSKPGALRRSLSRSMSQEAQRG
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal GST-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    3-7 business days
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

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