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MYH6 Antibody

  • 货号：
    CSB-PA015299HA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA015299HA01HU diluted at 1:100 and staining in paraffin-embedded human heart 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-PA015299HA01HU diluted at 1:100 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.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) MYH6 Polyclonal antibody
  • Uniprot No.：
    P13533
  • 基因名：
  • 别名：
    cardiac muscle alpha isoform antibody; MYH6 antibody; MYH6_HUMAN antibody; MYHC antibody; MyHC-alpha antibody; MyHC-beta antibody; MYHCA antibody; MYHCB antibody; Myosin heavy chain 6 antibody; Myosin heavy chain antibody; Myosin heavy chain cardiac muscle alpha isoform antibody; Myosin heavy chain cardiac muscle beta isoform antibody; Myosin-6 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Myosin-6 protein (894-1132AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，MYH6 Antibody (CSB-PA015299HA01HU)，的标记方式是Non-conjugated。对于MYH6 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-PA015299HB01HU MYH6 Antibody, HRP conjugated ELISA
    FITC CSB-PA015299HC01HU MYH6 Antibody, FITC conjugated
    Biotin CSB-PA015299HD01HU MYH6 Antibody, Biotin conjugated ELISA
  • 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：
    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
  • 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：
    Muscle contraction.
  • 基因功能参考文献：
    1. Three loci with high mutation frequencies, the 138665410 FOXL2 gene variant, the 23862952 MYH6 gene variant, and the 71098693 HYDIN gene variant were found to be significantly associated with sporadic Atrial Septal Defect (P<0.05); variants in FOXL2 and MYH6 were found in patients with isolated, sporadic Atrial Septal Defect (P<5x10-4). PMID: 29505555
    2. We developed an human cardiac alpha-myosin -induced myocarditis model in human HLA-DR4 transgenic mice that lack all mouse MHCII genes. PMID: 28431892
    3. The etiology of MYH6-associated HLHS can be informed using iPSCs. PMID: 27789736
    4. Rare inherited and de novo variants in 2,871 congenital heart disease probands identified GDF1, MYH6, and FLT4 as causative genes. PMID: 28991257
    5. the P830L and A1004S alphaMHC mutations alter myocyte contractility in completely different ways while at the same preserving peak intracellular calcium PMID: 28088328
    6. Data show that compound heterozygosity for recessive myosin heavy chain 6 (MYH6) mutations in patients with hypoplastic left heart and reduced systemic right ventricular ejection fraction. PMID: 26085007
    7. human alpha- and beta-cardiac myosin, as well as the mutants, show opposite mechanical and enzymatic phenotypes with respect to each other. PMID: 25937279
    8. The novel MYH6 mutation delE933 causes both structural damage of the sarcomere and functional impairments on atrial action propagation. PMID: 25717017
    9. Data from molecular dynamic/docking simulations suggest that actin-myosin binding free energy accepts contributions from both electrostatic and nonpolar forces; studies compare cardiac alpha-myosin, beta-myosin, and fast skeletal muscle myosin. PMID: 24224850
    10. R1165C mutation in MYH9 gene is associated with macroscopic hematuria and presenile cataract. PMID: 22627578
    11. Perturbations in the MYH6 head domain seem to play a major role in the genetic origin of familial Secundum-type atrial septal defects. PMID: 22194935
    12. the alpha-isoform of myosin heavy chain is the pathogenic autoantigen for CD4+ T cells in myocarditis PMID: 21436590
    13. the lifetime risk of being diagnosed with sick sinus syndrome is around 6% for non-carriers of c.2161C>T but is approximately 50% for carriers of the c.2161C>T variant PMID: 21378987
    14. data indicate that functional variants of MYH6 are associated with cardiac malformations in addition to atrial septal defect and provide a novel potential mechanism PMID: 20656787
    15. The mutations in MYH6 cause when a genetic cause can be identified, which has estimated to occur in 65% of hypertrophic cardiomyopathy. PMID: 20215591
    16. Our data provide evidence for a novel form of calcium-independent positive inotropy in failing cardiac myocytes by fast alpha-myosin motor protein gene transfer. PMID: 19801488
    17. results show that PUR proteins are capable of binding to alpha-MHC mRNA and attenuate its translational efficiency; also show robust expression of PUR proteins in failing hearts where alpha-MHC mRNA levels are suppressed PMID: 12933792
    18. Mutation in myosin heavy chain 6 causes atrial septal defect PMID: 15735645
    19. Three heterozygous MYH6 missense mutations were identified in dilated cardiomyopathy probands (P830L, A1004S, and E1457K; 4.3% of probands). A Q1065H mutation was detected in 1 of 21 hypertrophic cardiomyopathy probands. PMID: 15998695
    20. the large step of dimeric myosin VI is primarily made possible by a medial tail in each monomer that forms a rare single alpha-helix of approximately 10 nm, which is anchored to the calmodulin-bound IQ domain by a globular proximal tail. PMID: 18511944

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  • 相关疾病：
    Atrial septal defect 3 (ASD3); Cardiomyopathy, familial hypertrophic 14 (CMH14); Cardiomyopathy, dilated 1EE (CMD1EE); Sick sinus syndrome 3 (SSS3)
  • 亚细胞定位：
    Cytoplasm, myofibril. Note=Thick filaments of the myofibrils.
  • 蛋白家族：
    TRAFAC class myosin-kinesin ATPase superfamily, Myosin family
  • 数据库链接：

    HGNC: 7576

    OMIM: 160710

    KEGG: hsa:4624

    STRING: 9606.ENSP00000348634

    UniGene: Hs.278432