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ACTN2 Antibody

  • 货号：
    CSB-PA001242GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    P35609
  • 基因名：
    ACTN2
  • 别名：
    Actin binding protein antibody; Actinin alpha 2 antibody; ACTN 2 antibody; ACTN2 antibody; ACTN2_HUMAN antibody; Alpha actinin 2 antibody; Alpha actinin skeletal muscle antibody; Alpha actinin skeletal muscle isoform 2 antibody; Alpha-actinin skeletal muscle isoform 2 antibody; Alpha-actinin-2 antibody; CMD1AA antibody; F actin cross linking protein antibody; F-actin cross-linking protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human ACTN2
  • 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,WB
  • 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：
    F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
  • 基因功能参考文献：
    1. A dual-beam optical tweezers measured the mechanics of alpha-actinin 2 and rabbit titin interaction at the single-molecule level. Depending on the direction of force application, the unbinding forces can more than triple. Multiple alpha-actinin/Z-repeat interactions cooperate to ensure long-term stable titin anchoring, while allowing the individual components to exchange dynamically. PMID: 28096424
    2. These results provide new insights into the regulation of SK2 channel trafficking by the cytoskeletal proteins FLNA and alpha-actinin2, involving distinct recycling pathways PMID: 27779751
    3. the interaction between GNE and alpha-actinin 1 and alpha-actinin 2 occur at different sites in the alpha-actinin molecules and that for alpha-actinin 2 the interaction site is located at the C-terminus of the protein. PMID: 27023225
    4. The full length mEos2 tagged protein expressed in adult cardiomyocytes shows that both mutations additionally affect Z-disc localization and dynamic behaviour PMID: 27287556
    5. study strengthens the hypothesis that ACTN2 influences caries risk. PMID: 24810274
    6. The novel heterozygous missense sequence variant ACTN2 cosegregated with a complex cardiomyopathic trait, characterized by the interplay of midapical, nonobstructive HCM, early onset of AF and AV block, as well as regional LV noncompaction. PMID: 25173926
    7. Clinical evaluation of an Australian family revealed diverse cardiac pathologies in four affected members and genetic testing of the exome identified a pathogenic ACTN2 heterozygous variant (Ala119Thr) that co-segregated with disease. PMID: 25224718
    8. Study reports a complete high-resolution structure of the 200 kDa alpha-actinin-2 dimer from striated muscle and explore its functional implications on the biochemical and cellular level. PMID: 25433700
    9. This study generated the genomic sequences of K88-positive and F18-positive porcine enteroteoxigenic E. coli (ETEC) strains and examined the phylogenetic distribution of clinical porcine ETEC strains and their plasmid-associated genetic content. PMID: 22081385
    10. Findigs show that the F-actin-binding protein alpha-actinin-2 targets CaMKIIalpha to F-actin in cells by binding to the CaMKII regulatory domain. PMID: 22427672
    11. data provide functional evidence that the primary sequences of alpha-actinin-2 and alpha-actinin-3 evolved differences to optimize their functions PMID: 22253474
    12. This is the first genome-wide linkage analysis that shows mutations in ACTN2 cause HCM PMID: 20022194
    13. BPAG1-b was detectable in vitro and in vivo as a high molecular mass protein in striated and heart muscle cells, co-localizing with alpha-actinin-2 and partially with the cytolinker plectin as well as with the intermediate filament protein desmin. PMID: 19932097
    14. Spectrin-like repeats from dystrophin and alpha-actinin-2 are not functionally interchangeable. PMID: 12140183
    15. Association of Ca2+-activated K+ channel with alpha-actinin2 localizes channel to entry of external Ca(2+) source, which regulates channel function. PMID: 17110593
    16. actinin-2 participate in sequestering parafibromin in the cytoplasmic compartment. PMID: 18687124
    17. ACTN2 expression is affected by the content of alpha-actinin-3. PMID: 19150855
    18. demonstrate that proper membrane localization of a small-conductance Ca(2+)-activated K(+) channel (SK2 or K(Ca)2.2) is dependent on its interacting protein, alpha-actinin2, a major F-actin crosslinking protein. PMID: 19815520

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  • 相关疾病：
    Cardiomyopathy, familial hypertrophic 23, with or without left ventricular non-compaction (CMH23); Cardiomyopathy, dilated 1AA, with or without left ventricular non-compaction (CMD1AA)
  • 亚细胞定位：
    Cytoplasm, myofibril, sarcomere, Z line. Note=Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.
  • 蛋白家族：
    Alpha-actinin family
  • 组织特异性：
    Expressed in both skeletal and cardiac muscle.
  • 数据库链接：

    HGNC: 164

    OMIM: 102573

    KEGG: hsa:88

    STRING: 9606.ENSP00000355537

    UniGene: Hs.498178