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CSRP3 Antibody

  • 货号：
    CSB-PA006087ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: CSRP3 antibody at 1μg/ml + Mouse skeletal muscle tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 21 kDa
      Observed band size: 21, 25 kDa
    • Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA006087ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA006087ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) CSRP3 Polyclonal antibody
  • Uniprot No.：
    P50461
  • 基因名：
    CSRP3
  • 别名：
    cardiac antibody; Cardiac LIM protein antibody; CLP antibody; CMD1M antibody; CMH12 antibody; CRP3 antibody; Csrp3 antibody; CSRP3_HUMAN antibody; Cysteine and glycine-rich protein 3 antibody; Cysteine rich protein 3 antibody; Cysteine-rich protein 3 antibody; LIM domain only 4 antibody; LIM domain protein antibody; LMO4 antibody; MLP antibody; Muscle LIM protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Mouse
  • 免疫原：
    Recombinant Human Cysteine and glycine-rich protein 3 protein (1-194AA)
  • 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, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    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：
    Positive regulator of myogenesis. Acts as cofactor for myogenic bHLH transcription factors such as MYOD1, and probably MYOG and MYF6. Enhances the DNA-binding activity of the MYOD1:TCF3 isoform E47 complex and may promote formation of a functional MYOD1:TCF3 isoform E47:MEF2A complex involved in myogenesis. Plays a crucial and specific role in the organization of cytosolic structures in cardiomyocytes. Could play a role in mechanical stretch sensing. May be a scaffold protein that promotes the assembly of interacting proteins at Z-line structures. It is essential for calcineurin anchorage to the Z line. Required for stress-induced calcineurin-NFAT activation. The role in regulation of cytoskeleton dynamics by association with CFL2 is reported conflictingly: Shown to enhance CFL2-mediated F-actin depolymerization dependent on the CSRP3:CFL2 molecular ratio, and also shown to reduce the ability of CLF1 and CFL2 to enhance actin depolymerization. Proposed to contribute to the maintenance of muscle cell integerity through an actin-based mechanism. Can directly bind to actin filaments, cross-link actin filaments into bundles without polarity selectivity and protect them from dilution- and cofilin-mediated depolymerization; the function seems to involve its self-association. In vitro can inhibit PKC/PRKCA activity. Proposed to be involved in cardiac stress signaling by down-regulating excessive PKC/PRKCA signaling.; May play a role in early sarcomere organization. Overexpression in myotubes negatively regulates myotube differentiation. By association with isoform 1 and thus changing the CSRP3 isoform 1:CFL2 stoichiometry is proposed to down-regulate CFL2-mediated F-actin depolymerization.
  • 基因功能参考文献：
    1. Previous results along with the newly identified homozygous CSRP3 truncating variants in two unrelated hypertrophic cardiomyopathy (HCM) patients suggest that the association of CSRP3 as a validated HCM-causing gene require additional studies and those CSRP3 variants could result in HCM with an autosomal recessive inheritance rather than with an autosomal dominant transmission as usually reported on HCM. PMID: 30012424
    2. MLP contributes to the maintenance of cardiomyocyte cytoarchitecture by a mechanism involving its self-association and actin filament cross-linking. PMID: 24934443
    3. study reports the discovery of an alternative splice variant of muscle lim protein encoded by the CSRP3 gene, designated as MLP-b, showing distinct expression in neuromuscular disease and direct roles in actin dynamics and muscle differentiation PMID: 24860983
    4. KLF5 reverses hhLIM function from anti-proliferation to pro-proliferation through its interaction with hhLIM on the cyclin E promoter. PMID: 22584587
    5. The CSRP3-W4R mutation causes cardiomyopathy and heart failure in patients and engineered knock-in animals. PMID: 20044516
    6. CSRP3 is involved in cardiac mechanosensory processes, is localized to the sarcomeric Z-disc and human mutations cause cardiomyopathy(DCM)and heart failure. PMID: 12507422
    7. Mutations in the CRP3/MLP gene can cause hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). PMID: 12642359
    8. CSRP3, MUSTN1, SIX1, and FBXO32 expression changes in response to lengthening and shortening contractions in human muscle PMID: 17519359
    9. A myocardial actin-binding protein that increases actin cytoskeleton stability by promoting bundling of actin filaments. PMID: 18331358
    10. These findings suggest that hhLIM is a typical LIM family member with powerful transcription activation. PMID: 18393774
    11. Study used linkage analysis and identified a CSRP3 missense mutation in a large German family affected by hypertrophic cardiomyopathy. PMID: 18505755
    12. CSRP3 mutation was found involved in hypertrophic cardiomyopathy. PMID: 19035361
    13. The structure of both LIM domains of human MLP by nuclear magnetic resonance spectroscopy. PMID: 19230835
    14. CRP3/MLP is primarily expressed in arterial smooth muscle cells and that stretch is the main stimulus for CRP3/MLP induction in veins exposed to arterial haemodynamic conditions. PMID: 19351738
    15. Complete chemical shift assignment was achieved for the first LIM domain and for most of the second domain, the N-terminal and C-terminal linker and part of the intervening linker. PMID: 19636821
    16. MLP binds directly to CFL2 in human cardiac and skeletal muscles. PMID: 19752190

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  • 相关疾病：
    Cardiomyopathy, dilated 1M (CMD1M); Cardiomyopathy, familial hypertrophic 12 (CMH12)
  • 亚细胞定位：
    Nucleus. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm, myofibril, sarcomere.; [Isoform 2]: Cytoplasm, myofibril, sarcomere, Z line.
  • 组织特异性：
    Cardiac and slow-twitch skeletal muscles. Isoform 2 is expressed in striated muscle. Isoform 2 is specifically expressed at higher levels in patients with neuromuscular diseases, such as limb-girdle muscular dystrophy 2A (LGMD2A), Duchenne muscular dystro
  • 数据库链接：

    HGNC: 2472

    OMIM: 600824

    KEGG: hsa:8048

    STRING: 9606.ENSP00000265968

    UniGene: Hs.83577