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MYBPC1 Antibody

  • 货号：
    CSB-PA010297
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of 293 cells using MYBPC1 Polyclonal Antibody
  • 其他：

产品详情

  • 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,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human MYBPC1.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:20000
  • 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