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LDB3 Antibody

  • 货号：
    CSB-PA012831ESR2HU
  • 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-PA012831ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA012831ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) LDB3 Polyclonal antibody
  • Uniprot No.：
    O75112
  • 基因名：
    LDB3
  • 别名：
    CMD1C antibody; CYPHER antibody; HGNC:15710 antibody; KIAA01613 antibody; KIAA0613 antibody; Ldb3 antibody; LDB3_HUMAN antibody; LDB3Z1 antibody; LDB3Z4 antibody; LIM domain binding 3 antibody; LIM domain binding protein 3 antibody; LIM domain-binding protein 3 antibody; LVNC3 antibody; ORACLE antibody; PDLIM6 antibody; PDZ and LIM domain 6 antibody; Protein cypher antibody; Z band alternatively spliced PDZ motif antibody; Z band alternatively spliced PDZ motif protein antibody; Z-band alternatively spliced PDZ-motif protein antibody; ZASP antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human LIM domain-binding protein 3 protein (1-283AA)
  • 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：
    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