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MYBPC1 Antibody

  • 货号：
    CSB-PA717389LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA717389LA01HU diluted at 1:600 and staining in paraffin-embedded human skeletal muscle tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA717389LA01HU diluted at 1:600 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of A549 cells with CSB-PA717389LA01HU at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) MYBPC1 Polyclonal antibody
  • Uniprot No.：
    Q00872
  • 基因名：
    MYBPC1
  • 别名：
    C protein, skeletal muscle slow isoform antibody; C-protein antibody; MYBPC1 antibody; MYBPCC antibody; MYBPCS antibody; Myosin binding protein C, slow type antibody; Myosin-binding protein C antibody; MYPC1_HUMAN antibody; skeletal muscle C protein antibody; skeletal muscle slow isoform antibody; Slow MyBP C antibody; Slow MyBP-C antibody; slow-type antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Myosin-binding protein C, slow-type protein (618-779AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，MYBPC1 Antibody (CSB-PA717389LA01HU)，的标记方式是Non-conjugated。对于MYBPC1 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA717389LB01HU MYBPC1 Antibody, HRP conjugated ELISA
    FITC CSB-PA717389LC01HU MYBPC1 Antibody, FITC conjugated
    Biotin CSB-PA717389LD01HU MYBPC1 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:500-1:1000
    IF 1:200-1:500
  • 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：
    Thick filament-associated protein located in the crossbridge region of vertebrate striated muscle a bands. Slow skeletal protein that binds to both myosin and actin. In vitro, binds to native thin filaments and modifies the activity of actin-activated myosin ATPase. May modulate muscle contraction or may play a more structural role.
  • 基因功能参考文献：
    1. A novel milder MYBPC1 homozygous phenotype causes arthrogryposis multiplex congenita in a consanguineous Israeli Druze pedigree. PMID: 26661508
    2. Ca(2+) modulates the interaction of cMyBP-C with the thin filament in the sarcomere. PMID: 26831109
    3. Two novel mutations in myosin binding protein C slow causing distal arthrogryposis type 2 were both found to occur in the C2 immunoglobulin domain, which constitutes part of the binding site for the S2 subfragment of myosin. PMID: 25679999
    4. Mutations in the MYH7 gene, rather than in the MYBPC3 gene, were also related to a worse prognosis. This is the first work characterizing HC molecular epidemiology in the Brazilian population for the 3 most important genes. PMID: 24093860
    5. Autosomal recessive lethal congenital contractural syndrome type 4 (LCCS4) caused by a mutation in MYBPC1 PMID: 22610851
    6. Significant molecule MYBPC1 phosphoprotein network from 12 frontal cortex of HIV encephalitis (HIVE) control patients and 16 HIVE, was identified and constructed. PMID: 21061152
    7. MyBPC1 acts as an adaptor to connect the ATP consumer (myosin) and the regenerator (muscle type creatine kinase) for efficient energy metabolism and homoeostasis. PMID: 21426302
    8. These findings reveal that the MYBPC1 is a novel gene responsible for DA1, though the mechanism of disease may differ from how some cardiac MYBPC3 mutations cause hypertrophic cardiomyopathy. PMID: 20045868
    9. Screening patients with dilated cardiomyopathy, as well as hypertrophic cardiomyopathy, for this mutation is of signifiant importance with this mutation diagnosing dilated cardiomyopathy. PMID: 12628722
    10. The present study demonstrates slow skeletal muscle type C-protein in moderate amount in right atrium and interatrial septum of adult human, rabbit, rat and bovine hearts using both immunocytochemical and immunoblotting procedures. PMID: 16003462
    11. to determine whether HCM mutations in beta myosin heavy chain located within the light meromyosin portion alter the binding of cMyBP-C, and to define the precise region of this binding. PMID: 16918501

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  • 相关疾病：
    Arthrogryposis, distal, 1B (DA1B); Lethal congenital contracture syndrome 4 (LCCS4)
  • 蛋白家族：
    Immunoglobulin superfamily, MyBP family
  • 数据库链接：

    HGNC: 7549

    OMIM: 160794

    KEGG: hsa:4604

    STRING: 9606.ENSP00000354849

    UniGene: Hs.654589