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  4. ACTA1 Recombinant Monoclonal Antibody

ACTA1 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA001205A0HU
  • 规格：
    ￥1320
  • 图片：
    • Western Blot
      Positive WB detected in: Hela whole cell lysate, A549 whole cell lysate, Raw264.7 whole cell lysate, NIH/3T3 whole cell lysate, HepG2 whole cell lysate, Rat brain tissue, Rat heart tissue
      All lanes: Actin antibody at 0.95μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 42 KDa
      Observed band size: 42 KDa
    • IHC image of CSB-RA001205A0HU diluted at 1:100 and staining in paraffin-embedded human kidney 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℃ overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of HepG2 cells with CSB-RA001205A0HU at 1:60,counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4℃.The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG (H+L).
    • Overlay histogram showing Hela cells stained with CSB-RA001205A0HU (red line) at 1:50. The cells were fixed with 70% Ethylalcohol (18h) and then permeabilized with 0.3% Triton X-100 for 2 min. The cells were then incubated in 1x PBS /10% normal goat serum to block non-specific protein-protein interactions followed by primary antibody for 1 h at 4℃. The secondary antibody used was FITC goat anti-rabbit IgG (H+L) at 1/200 dilution for 1 h at 4℃. Control antibody (green line) was used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

产品详情

  • 产品描述：

    CUSABIO cloned the DNA sequence encoding the ACTA1 monoclonal antibody into the plasmid and then transfected into the cell line for expression. The product was purified through the affinity-chromatography method and then got the recombinant ACTA1 monoclonal antibody. It belongs to the rabbit IgG. This ACTA1 antibody is reactive with human ACTA1 protein and has been validated in ELISA, WB, IHC, IF, and FC applications.

    ACTA1 is a significant component in the skeletal muscle thin filament of the sarcomere, and it is required for force production, muscle contraction, and movement. So it is linked to a number of muscle disorders. In mice, knocking out ACTA1 results in muscular weakness and death in the early neonatal period. Congenital myopathies such as nemaline myopathy (NM), intranuclear rod myopathy (IRM), and actin myopathy (AM) are caused by amino acid changes in the ACTA1 protein.

  • Uniprot No.：
    P68133
  • 基因名：
    ACTA1
  • 别名：
    a actin antibody; ACTA antibody; ACTA1 antibody; ACTA2 antibody; ACTC antibody; ACTC1 antibody; Actin antibody; ACTS_HUMAN antibody; ACTSA antibody; Alpha 2 actin antibody; alpha skeletal muscle antibody; Alpha-actin-1 antibody; Cardiac muscle alpha actin 1 antibody; Skeletal muscle alpha actin 1 antibody
  • 反应种属：
    Human, Mouse, Rat
  • 免疫原：
    A synthesized peptide
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    25E3
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC, IF, FC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:50-1:500
    IF 1:30-1:200
  • 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：
    Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
  • 基因功能参考文献：
    1. Our study demonstrates that high ActA level is an independent prognosis factor of survival in cancer patients. More than a basic marker of the severity of the neoplastic disease or of the inflammatory process, ActA seems to influence survival by contributing to the development of cachexia and loss of skeletal muscle mass. PMID: 28712119
    2. The study confirmed ACTA1 mutations in four patients, including one with intranuclear rods, one with large intracytoplasmic aggregates, and two with nemaline intracytoplasmic rods. PMID: 28780987
    3. Study shows that clinically severe ACTA1-related myopathy can present with muscle morphological findings suggestive of cytoplasmic body myopathy in the absence of definite nemaline rods. PMID: 28416349
    4. Shorter than normal thin filament length contributes to the impaired force generation in patients with thin filament myopathy, but only in those who harbor specific mutations in NEB or ACTA1. PMID: 27074222
    5. Over-expression of TNC, SMA, and vimentin were significantly correlated with the lower overall survival in prostate cancer patients. PMID: 28341124
    6. ANA and ASMA evaluation in patients with liver transplantation and no history of autoimmune disease has no clinical relevance, since it varies in time and is not related to any risk factors or liver injury. Routine autoimmunity evaluation should be avoided. PMID: 28337446
    7. Upon actin engagement, the N-terminal "strap" and helix 1 are displaced from the vinculin tail helical bundle to mediate actin bundling. PMID: 26493222
    8. This study reported the new information on the frequency and phenotypes of congenital myopathy caused by ACTA1 mutations in subjects >/=5 years of age. PMID: 26172852
    9. The s propose that Lpd delivers Ena/VASP proteins to growing barbed ends and increases their actin polymerase activity by tethering them to actin filaments. PMID: 26295568
    10. Mutations in ACTA1 can cause pathologic features consistent with myofibrillar myopathy. PMID: 25913210
    11. TIMP-1 significantly increased levels of alpha-SMA. PMID: 25895516
    12. novel homozygous recessive missense variant (c.460G>C, P.(Val154Leu)) was found in two brothers with infantile-onset congenital muscular dystrophy with rigid spine. PMID: 25182138
    13. Myopathy associated with zebra bodies is part of the spectrum of myopathies associated with the ACTA1 gene. PMID: 25747004
    14. New scapuloperoneal phenotype associated with an ACTA1 mutation. PMID: 25938801
    15. Antisynthetase syndrome-associated myositis is characterized by distinctive myonuclear actin filament inclusions. PMID: 25746564
    16. The predominant pathway mediated by Australian bat lyssavirus G envelope for internalization into HEK293T cells is clathrin-and actin-dependent also requiring Rab5. PMID: 24576301
    17. Data show that the width and speed of the waves depend counter-intuitively on parameters, negative feedback, and the F-actin time scale. PMID: 23831272
    18. Although most cases of severe infantile form of nemaline myopathy caused by ACTA1 mutations are sporadic and have no family history PMID: 24313005
    19. These findings suggest that the direct contact of Dengue virus 2 E protein with 43 kDa actin protein may have a crucial function in Dengue virus 2 infection of ECV304 cells. PMID: 23376163
    20. Myotonia in adult human skeletal actin transgenic mice may be explained on the basis of a mosaic expression of ClC-1 channels in different fibres and/or on alterations of other conductances. PMID: 23247112
    21. Respiratory muscles from an animal model of nemaline myopathy with an ACTA1 mutation show distinct muscle fiber weakness compared to limb muscles. PMID: 23656990
    22. The objective was to evaluate the presence and distribution of the lubricating and anti-adhesion glycoprotein lubricin and cells containing the contractile isoform smooth muscle alpha-actin (SMA) in pseudomembranes around loose hip prostheses. PMID: 23174700
    23. analysis of the pattern of of evolutionarily conserved basic and acidic residues that constitutes the binding interface of actin-tropomyosin PMID: 23420843
    24. Data show that GnRHR activation affected several cellular markers of locomotion, including actin organization and polymerization as well as active RhoA-GTP levels. PMID: 23176180
    25. Data indicate that actin nucleation on Chlamydia inclusions exhibited moderate depolymerization dynamics. PMID: 23071671
    26. The organization of actin and cytokeratin cytoskeleton and the expression of TCTP, p53,cyclin A, RhoA and actin in HIO180 non-transformed ovarian epithelial cells, and OVCAR3 and SKOV3 ovarian epithelial cancer cells, was studied. PMID: 23042265
    27. Data show that the urinary messenger RNA (mRNA) levels of alpha-smooth muscle actin (alpha-SMA), fibronectin, and matrix metalloproteinase-9 (MMP-9) were significantly higher in the diabetic nephropathy (DN) group, and mRNA levels increased with DN progression. PMID: 21824169
    28. High alpha-actin is associated with colorectal carcinogenesis. PMID: 21912905
    29. Over-expression of Nkx2.5 and/or cardiac alpha-actin inhibit the contraction ability of adipose tissue-derived stromal cells-derived cardiomyocytes. PMID: 21691712
    30. Platelets gal-1 forms an intracellular complex with monomeric actin. PMID: 22081313
    31. Herpesviruses exploit actin and actin-associated myosin motors for viral entry and virion egress. [review] PMID: 21994736
    32. CK2 regulates vaccinia virus dissemination and actin tail formation. PMID: 22209233
    33. Actin playes differential roles in trafficking of Epstein-Barr virus in B cells and epithelial cells. PMID: 22031939
    34. a link between extramuscular expression of alpha-skeletal muscle actin and clinical symptoms in non-skeletal muscle tissues of patients with ACTA1 mutations, and probably a functional role of alpha-skeletal muscle actin during fetal development PMID: 21514153
    35. The filopodium: a stable structure with highly regulated repetitive cycles of elongation and persistence depending on the actin cross-linker fascin PMID: 21975552
    36. Actin accumulates throughout the activated immunological synapse. PMID: 21931536
    37. Actin-EGFP and ezrin-EGFP accumulated below pilus-coated beads when force was applied. PMID: 21340023
    38. Data show that concurrent phosphorylation of cortactin by ERK1/2 and tyrosine kinases enables cells with the ability to regulate actin dynamics. PMID: 21079800
    39. BCL2 interaction with actin in vitro may inhibit cell motility by enhancing actin polymerization PMID: 20716950
    40. Results suggest that actin polymerization and bundling by VASP are critical for spine formation, expansion, and modulating synaptic strength. PMID: 20826790
    41. Centrosomal actin was detected with the anti-actin antibody 1C7 that recognizes antiparallel ("lower dimer") actin dimers. PMID: 21108927
    42. These findings demonstrated that PI3K-mediated actin rearrangements are required for Cronobacter sakazakii invasion of human brain microvascular endothelial cells. PMID: 20809254
    43. Results confirm that DNaseI-binding loop (D-loop) is involved in stabilization of skeletal muscle actin structure, both as monomers and filaments; actin is stabilized by ligands (by phalloidin, aluminum fluoride, & ATP [and to lesser extent by ADP]). PMID: 20718862
    44. Simultaneous tracking of both TCR clusters and GFP-actin speckles reveals their dynamic association and individual flow patterns. Actin retrograde flow directs the inward transport of TCR clusters PMID: 20686692
    45. We conclude that the Drosophila indirect flight muscles provide a good model system for studying ACTA1 mutations PMID: 20452215
    46. Data demonstrate for the first time that L-plastin contributes to the fine-tuning of actin turn-over, an activity which is regulated by Ser5 phosphorylation promoting its high affinity binding to the cytoskeleton. PMID: 20169155
    47. Podocyte BK(Ca) channels are regulated by synaptopodin, Rho, and actin microfilaments. PMID: 20630939
    48. these data identify NHS as a new regulator of actin remodelling. PMID: 20332100
    49. ACTA1 mutations are involved in fiber size disproportion in congenital myotonic dystrophy PMID: 20179953
    50. First insights of S100A10 function as a regulator of the filamentous actin network. PMID: 20100475

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  • 相关疾病：
    Nemaline myopathy 3 (NEM3); Myopathy, actin, congenital, with excess of thin myofilaments (MPCETM); Myopathy, congenital, with fiber-type disproportion (CFTD); Myopathy, scapulohumeroperoneal (SHPM)
  • 亚细胞定位：
    Cytoplasm, cytoskeleton.
  • 蛋白家族：
    Actin family
  • 数据库链接：

    HGNC: 129

    OMIM: 102610

    KEGG: hsa:58

    STRING: 9606.ENSP00000355645

    UniGene: Hs.1288