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      ACTN2 Antibody

      Datasheet
      • 貨號:
        CSB-PA001242LA11HU
      • 規格:
        ¥1320
      • 圖片:
        • Western Blot
          Positive WB detected in: Hela whole cell lysate, 293 whole cell lysate, Rat Brain tissue
          All lanes: ACTN2 antibody at 1:2000
          Secondary
          Goat polyclonal to rabbit IgG at 1/50000 dilution
          Predicted band size: 104 kDa
          Observed band size: 104 kDa
        • IHC image of CSB-PA001242LA11HU diluted at 1:500 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
      • 其他:

      產品詳情

      • 產品名稱:
        Rabbit anti-Homo sapiens (Human) ACTN2 Polyclonal antibody
      • Uniprot No.:
      • 基因名:
        ACTN2
      • 別名:
        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
      • 反應種屬:
        Human, Rat
      • 免疫原:
        Recombinant Human Alpha-actinin-2 protein (1-301AA)
      • 免疫原種屬:
        Homo sapiens (Human)
      • 標記方式:
        Non-conjugated
      • 克隆類型:
        Polyclonal
      • 抗體亞型:
        IgG
      • 純化方式:
        >95%, Protein G purified
      • 濃度:
        It differs from different batches. Please contact us to confirm it.
      • 保存緩沖液:
        Preservative: 0.03% Proclin 300
        Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
      • 產品提供形式:
        Liquid
      • 應用范圍:
        ELISA, WB, IHC
      • 推薦稀釋比:
        Application Recommended Dilution
        WB 1:1000-1:5000
        IHC 1:100-1:500
      • Protocols:
      • 儲存條件:
        Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
      • 貨期:
        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.

      靶點詳情

      • 功能:
        F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
      • 基因功能參考文獻:
        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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      • 相關疾?。?/div>
        Cardiomyopathy, familial hypertrophic 23, with or without left ventricular non-compaction (CMH23); Cardiomyopathy, dilated 1AA, with or without left ventricular non-compaction (CMD1AA)
      • 亞細胞定位:
        Cytoplasm, myofibril, sarcomere, Z line
      • 蛋白家族:
        Alpha-actinin family
      • 組織特異性:
        Expressed in both skeletal and cardiac muscle.
      • 數據庫鏈接:

        HGNC: 164

        OMIM: 102573

        KEGG: hsa:88

        STRING: 9606.ENSP00000355537

        UniGene: Hs.498178



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