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  4. Recombinant Human LIM domain-binding protein 3 (LDB3)

Recombinant Human LIM domain-binding protein 3 (LDB3)

  • 中文名称：
    人LDB3重组蛋白
  • 货号：
    CSB-EP012831HU
  • 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：
    LDB3
  • Uniprot No.：
  • 别名：
    CMD1C; CYPHER; HGNC:15710; KIAA01613; KIAA0613; Ldb3; LDB3_HUMAN; LDB3Z1; LDB3Z4; LIM domain binding 3; LIM domain binding protein 3; LIM domain-binding protein 3; LVNC3; ORACLE; PDLIM6; PDZ and LIM domain 6; Protein cypher; Z band alternatively spliced PDZ motif; Z band alternatively spliced PDZ motif protein; Z-band alternatively spliced PDZ-motif protein; ZASP
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Isoform 6
  • 来源：
    E.coli
  • 分子量：
    58.0kDa
  • 表达区域：
    1-283aa
  • 氨基酸序列
    MSYSVTLTGPGPWGFRLQGGKDFNMPLTISRITPGSKAAQSQLSQGDLVVAIDGVNTDTMTHLEAQNKIKSASYNLSLTLQKSKRPIPISTTAPPVQTPLPVIPHQKVVVNSPANADYQERFNPSALKDSALSTHKPIEVKGLGGKATIIHAQYNTPISMYSQDAIMDAIAGQAQAQGSDFSGSLPIKDLAVDSASPVYQAVIKSQNKPEDEADEWARRSSNLQSRSFRILAQMTGTEFMQDPDEEALRRSRERFETERNSPRFAKLRNWHHGLSAQILNVKS
    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：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 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：
    May function as an adapter in striated muscle to couple protein kinase C-mediated signaling via its LIM domains to the cytoskeleton.
  • 基因功能参考文献：
    1. Interaction is demonstrated between ZASP and two new binding partners both of which have roles in signalling, regulation of gene expression and muscle differentiation; the mechanosensing protein Ankrd2 and the tumour suppressor protein p53. PMID: 24647531
    2. p.(D117N) variant in Cypher/ZASP is not a causative mutation for dilated cardiomyopathy and ventricular arrhythmias. PMID: 26419279
    3. A novel heterozygous missense mutation (p.N155H) in a highly conserved PDZ-like motif of ZASP was identified. The results indicate that typical ZASP-MFM presenting with late-onset distal myopathy is commonly associated with mutations in PDZ-like motif of ZASP. PMID: 27546599
    4. The molecular basis for high-affinity binding of ZASP to G-Actin has been described. PMID: 28349680
    5. this is the first family with Arrhythmogenic right ventricular cardiomyopathy where a mutation in LDB3 is associated with Arrhythmogenic right ventricular cardiomyopathy PMID: 25041374
    6. Study identified abnormal inclusion of LDB3 exon 11 specific to myotonic dystrophy type at the RNA and protein level; inclusion changed the affinity of LDB3 for PKC, indicating that exon 11 may contribute to the activation of PKC in DM1 PMID: 24878509
    7. results show that MFM-associated ZASP mutations in the actin-binding domain have deleterious effects on the core structure of the Z-discs in skeletal muscle. PMID: 24668811
    8. Z-band alternatively spliced PDZ motif protein (ZASP) is the major O-linked beta-N-acetylglucosamine-substituted protein in human heart myofibrils PMID: 23271734
    9. This results of this indicted that One patient harbored the missense mutation c.1719G>A (p.V566M) in the ZASP gene. PMID: 22349865
    10. ZASP1 can form protein complex with telethonin/T-Cap and Na(v)1.5. The left ventricular noncompaction-specific ZASP1 mutation can cause loss of function of Na(v)1.5, without significant alteration of the cytoskeletal protein complex. PMID: 22929165
    11. TNNT3 and LDB3 showed abnormal splicing and appeared more pronounced in myotonic dystrophies type 2 relative to myotonic dystrophies type 1. PMID: 20066428
    12. the D626N mutation of Cypher/ZASP increases the affinity of the LIM domain for protein kinase C, which may be related to pathogenesis of dilated cardiomyopathy PMID: 14660611
    13. ZASP/Cypher internal fragments containing either ZM exon 4 or 6 co-localized with alpha-actinin in cultured myoblasts and nonmuscle cells. PMID: 16476425
    14. Tafazzins are essential during fetal and early post-natal life. PMID: 17394203
    15. ZASP/Cypher anchors PGM1 to Z-disc under conditions of stress. The impaired binding of PGM1 to ZASP/Cypher might be involved in the pathogenesis of dilated cardiomyopathy. PMID: 19377068

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  • 相关疾病：
    Cardiomyopathy, dilated 1C, with or without left ventricular non-compaction (CMD1C); Left ventricular non-compaction 3 (LVNC3); Myopathy, myofibrillar, 4 (MFM4)
  • 亚细胞定位：
    Cytoplasm, perinuclear region. Cell projection, pseudopodium. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=Localized to the cytoplasm around nuclei and pseudopodia of undifferentiated cells and detected throughout the myotubes of differentiated cells. Colocalizes with ACTN2 at the Z-lines.
  • 组织特异性：
    Expressed primarily in skeletal muscle and to a lesser extent in heart. Also detected in brain and placenta.
  • 数据库链接：

    HGNC: 15710

    OMIM: 601493

    KEGG: hsa:11155

    UniGene: Hs.657271