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  4. JPH2 Antibody

JPH2 Antibody

  • 中文名称：
    JPH2兔多克隆抗体
  • 货号：
    CSB-PA011964GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9BR39
  • 基因名：
    JPH2
  • 别名：
    FLJ40969 antibody; JP-2 antibody; JP2 antibody; Jph2 antibody; JPH2_HUMAN antibody; Junctophilin 2 antibody; Junctophilin type 2 antibody; Junctophilin-2 antibody; OTTHUMP00000031651 antibody; OTTHUMP00000031652 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Human Jph2
  • 免疫原种属：
    Homo sapiens (Human)
  • 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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 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：
    Membrane-binding protein that provides a structural bridge between the plasma membrane and the sarcoplasmic reticulum and is required for normal excitation-contraction coupling in cardiomyocytes. Provides a structural foundation for functional cross-talk between the cell surface and intracellular Ca(2+) release channels by maintaining the 12-15 nm gap between the sarcolemma and the sarcoplasmic reticulum membranes in the cardiac dyads. Necessary for proper intracellular Ca(2+) signaling in cardiac myocytes via its involvement in ryanodine receptor-mediated calcium ion release. Contributes to the construction of skeletal muscle triad junctions.; Transcription repressor required to safeguard against the deleterious effects of cardiac stress. Generated following cleavage of the Junctophilin-2 chain by calpain in response to cardiac stress in cardiomyocytes. Following cleavage and release from the membrane, translocates to the nucleus, binds DNA and represses expression of genes implicated in cell growth and differentiation, hypertrophy, inflammation and fibrosis. Modifies the transcription profile and thereby attenuates pathological remodeling in response to cardiac stress. Probably acts by competing with MEF2 transcription factors and TATA-binding proteins.
  • 基因功能参考文献：
    1. a novel mutation in JPH2 which suggests for the first time that JPH2 could cause dilated cardiomyopathy. PMID: 27471098
    2. Junctophilin-2 is cleaved by calpain at multiple sites, resulting in dysfunctional junctophilin-2 truncations. PMID: 26063807
    3. Our data identify a mechanistic link between microtubule densification and T-tubule remodeling and reveal microtubule-mediated JP2 redistribution as a novel mechanism for T-tubule disruption, loss of excitation-contraction coupling, and heart failure. PMID: 24519927
    4. The S101R mutation may have an effect upon the stability of the JP2 dyad organization with the potential to alter JP2-protein interactions regulating calcium cycling. PMID: 24001019
    5. Hypertrophy in skeletal myotubes induced by junctophilin-2 mutant, Y141H, involves an increase in store-operated Ca2+ entry via Orai1. PMID: 22389502
    6. Loss-of-function mutations in JPH2 leads to cardiac-specific JPH2 deficiency and results in impaired cardiac contractility, causing heart failure and increased mortality. PMID: 21339484
    7. JPH2 levels are reduced in patients with hypertrophic cardiomyopathy. Reduced JPH2 expression results in reduced excitation-contraction coupling gain as well as altered Ca(2+) homeostasis, which may be associated with prohypertrophic remodeling. PMID: 21216834
    8. S165F mutation of junctophilin 2 affects Ca2+ signalling in skeletal muscle. PMID: 20095964
    9. this is the first report on JPH2 mutation associated with hypertrophic cardiomyopathy PMID: 17476457
    10. Mutations in JPH2-encoded junctophilin-2 associated with hypertrophic cardiomyopathy. PMID: 17509612

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  • 相关疾病：
    Cardiomyopathy, familial hypertrophic 17 (CMH17)
  • 亚细胞定位：
    [Junctophilin-2]: Cell membrane; Peripheral membrane protein. Sarcoplasmic reticulum membrane; Single-pass type IV membrane protein. Endoplasmic reticulum membrane; Single-pass type IV membrane protein.; [Junctophilin-2 N-terminal fragment]: Nucleus.
  • 蛋白家族：
    Junctophilin family
  • 组织特异性：
    Specifically expressed in skeletal muscle and heart.
  • 数据库链接：

    HGNC: 14202

    OMIM: 605267

    KEGG: hsa:57158

    STRING: 9606.ENSP00000362071

    UniGene: Hs.441737