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  4. ACTN2 Recombinant Monoclonal Antibody

ACTN2 Recombinant Monoclonal Antibody

  • 中文名稱：
    ACTN2 Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA972552A0HU
  • 規格：
    ￥1320
  • 圖片：
    • IHC image of CSB-RA972552A0HU 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA972552A0HU diluted at 1:100 and staining in paraffin-embedded human gastric cancer 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.
    • Overlay Peak curve showing MKN45 cells stained with CSB-RA972552A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    ACTN2
  • 別名：
    Alpha-actinin-2 (Alpha-actinin skeletal muscle isoform 2) (F-actin cross-linking protein), ACTN2
  • 反應種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from Human ACTN2 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    13F2
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:50-1:200
    FC 1:20-1:200
  • 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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

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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  • 相關疾病：
    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. Note=Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.
  • 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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