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PDGFRA Antibody

  • 货号：
    CSB-PA030000
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    P16234
  • 基因名：
  • 别名：
    Alpha-type platelet-derived growth factor receptor antibody; CD140 antigen-like family member A antibody; CD140a antibody; CD140a antigen antibody; MGC74795 antibody; PDGF alpha chain antibody; PDGF Receptor alpha antibody; PDGF-R-alpha antibody; PDGFR 2 antibody; PDGFR alpha antibody; PDGFR2 antibody; PDGFRA antibody; PDGFRA/BCR fusion antibody; PGFRA_HUMAN antibody; Platelet derived growth factor receptor 2 antibody; Platelet derived growth factor receptor alpha antibody; Platelet derived growth factor receptor alpha polypeptide antibody; Platelet derived growth factor receptor antibody; Rearranged in hypereosinophilia platelet derived growth factor receptor alpha fusion protein antibody; RHEPDGFRA antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from Human PDGFR-α around the non-phosphorylation site of Y1018.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:5000
  • 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：
    Tyrosine-protein kinase that acts as a cell-surface receptor for PDGFA, PDGFB and PDGFC and plays an essential role in the regulation of embryonic development, cell proliferation, survival and chemotaxis. Depending on the context, promotes or inhibits cell proliferation and cell migration. Plays an important role in the differentiation of bone marrow-derived mesenchymal stem cells. Required for normal skeleton development and cephalic closure during embryonic development. Required for normal development of the mucosa lining the gastrointestinal tract, and for recruitment of mesenchymal cells and normal development of intestinal villi. Plays a role in cell migration and chemotaxis in wound healing. Plays a role in platelet activation, secretion of agonists from platelet granules, and in thrombin-induced platelet aggregation. Binding of its cognate ligands - homodimeric PDGFA, homodimeric PDGFB, heterodimers formed by PDGFA and PDGFB or homodimeric PDGFC -leads to the activation of several signaling cascades; the response depends on the nature of the bound ligand and is modulated by the formation of heterodimers between PDGFRA and PDGFRB. Phosphorylates PIK3R1, PLCG1, and PTPN11. Activation of PLCG1 leads to the production of the cellular signaling molecules diacylglycerol and inositol 1,4,5-trisphosphate, mobilization of cytosolic Ca(2+) and the activation of protein kinase C. Phosphorylates PIK3R1, the regulatory subunit of phosphatidylinositol 3-kinase, and thereby mediates activation of the AKT1 signaling pathway. Mediates activation of HRAS and of the MAP kinases MAPK1/ERK2 and/or MAPK3/ERK1. Promotes activation of STAT family members STAT1, STAT3 and STAT5A and/or STAT5B. Receptor signaling is down-regulated by protein phosphatases that dephosphorylate the receptor and its down-stream effectors, and by rapid internalization of the activated receptor.
  • 基因功能参考文献：
    1. Hepatic stellate cells release PDGFRalpha-enriched extracellular vesicles. Alcoholic liver disease patients show an increase in PDGFRalpha enrichment in their serum extracellular vesicles. PMID: 29360139
    2. In addition to replicating a previously identified genome-wide significant locus for corneal astigmatism near the PDGFRA gene, gene-based analysis identified three novel candidate genes, CLDN7, ACP2, and TNFAIP8L3, that warrant further investigation to understand their role in the pathogenesis of corneal astigmatism. (Meta-analysis) PMID: 29422769
    3. The present study showed that PDGFRA amplification could be effectively targeted by pazopanib. PMID: 30060824
    4. KIT and PDGFRA mutations account for 85-90% of GISTs; subsequent genetic studies have led to the identification of mutation/epimutation of additional genes, including the succinate dehydrogenase (SDH) subunit A, B, C, and D genes. PMID: 29413424
    5. we compared the efficacy of first-line therapy, doxorubicin (DOX), and TRAB in a platelet-derived growth factor receptor-alpha (PDGFRA)-amplified PLPS. METHODS: We used a fresh sample of PLPS tumor derived from a 68-year-old male patient diagnosed with a recurrent Pleomorphic liposarcoma PMID: 30126369
    6. PDGFRA D842V mutant binds imatinib with lower affinity with respect to wild-type structure, showing higher stability during the interaction with other type I TKIs (like crenolanib). PMID: 29510530
    7. Altered SK3 channel expression observed in PDGFRalpha(+) cells in UPJ obstruction suggests that the impairment of SK3 activity across the UPJ may perturb upper urinary tract peristalsis in this urological condition PMID: 28902181
    8. None of the 16 analyzable tumors showed mutations in PDGFRA. Thus, PDGFRA mutations probably do not play an important role in the development of sporadic lipomas of the intestines PMID: 26990750
    9. Whole transcriptome sequencing followed by pathway analysis indicated that STXBP4 is involved in functional gene networks that regulate cell growth, proliferation, cell death, and survival in cancer. Platelet-derived growth factor receptor alpha (PDGFRalpha) was a key downstream mediator of STXBP4 function. In line with this, shRNA mediated STXBP4 and PDGFRA knockdown suppressed tumor growth in soft-agar and xenograft ... PMID: 28087642
    10. We report a unique case of an SDH-deficient GIST case with an activating PDGFRA mutation. Oncogenic mutations in GIST are generally mutually exclusive; however documented exceptions exist which may have diagnostic and therapeutic implications. PMID: 28768491
    11. Case Report: concurrent development of myeloproliferative hypereosinophilic syndrome and lymphomatoid papulosis associated with FIP1L1-PDGFRA gene fusion. PMID: 28374041
    12. Our results suggest that PDGFRalpha overexpression in HCC is a prognostic marker independent of adjacent non-tumor site liver fibrosis status. PMID: 28465473
    13. PDGFRalpha/PDGFRbeta signaling balance determines progenitor commitment to beige (PDGFRalpha) or white (PDGFRbeta) adipogenesis. PMID: 29158445
    14. overview of primary cilia-mediated regulation of receptor tyrosine kinase (RTK- PDGFRa and PDGFRb) and transforming growth factor beta (TGF-beta) signaling [review] PMID: 27638178
    15. PDGFRA mutation, but not amplification is associated with older age in pediatric high-grade glioma. PMID: 27582545
    16. Study demonstrates for the first time that PDGFR-alpha strongly inhibits endothelial and melanoma cells proliferation in a CXCL10/IP-10 dependent way, via miR-503 down-regulation. PMID: 27764787
    17. PDGFRalpha activation is an essential component that drives aggressiveness in papillary thyroid carcinoma cells. The signaling pathways are complex, involving not only the MAPK/Erk but also the PI3K/Akt and STAT3 pathways. PMID: 27845909
    18. Here, the s provide a 19 A reconstruction for the cytomegalovirus gHgLgO trimer and show that it binds with high affinity through the gO subunit to PDGFRalpha, which is expressed on fibroblasts but not on epithelial cells. PMID: 27573107
    19. Perivascular PDGFR-alpha and -beta were identified as independent markers predicting survival in metastatic colorectal cancer (mCRC). PMID: 27248825
    20. Data suggest that the platelet derived growth factor receptor alpha (PDGFRalpha)/Stat3 transcription factor/Rb1 protein regulatory axis might represent a potential therapeutic target for glioblastoma (GBM) treatment. PMID: 27344175
    21. Point mutations in the PDGFRa gene, which leads to amino acid residue changes activating the kinase of the receptor, occur in about 5% of Gastrointestinal Stroma Tumors. An activating deletion mutation of the PDGFRA gene has been described in a human Glioblastoma. PMID: 28940884
    22. FIP1L1/ PDGFRA associated chronic eosinophilic leukemia has an excellent long-term prognosis following imatinib therapy. PMID: 27120808
    23. Olaratumab had an acceptable adverse event profile in patients with gastrointestinal stromal tumor (GIST). While there was no apparent effect on PFS in patients without PDGFRa mutations, patients with PDGFRalpha-mutant GIST (all with D842V mutations) treated with olaratumab had longer disease control compared with historical data for this genotype PMID: 28426120
    24. For hot spots in KIT and PDGFRA genes, 23 out of 146 KIT/PDGFRA wild-type cases carried mutations according to next-generation sequencing (NGS). PMID: 26848617
    25. In vitro activation of PDGFR-alpha leads to translational activation of LAMB1, which in turn induces an invasive and metastatic phenotype of hepatocellular carcinoma cells exhibiting K19 expression. PMID: 28783171
    26. PDGFRalpha levels are regulated by SMARCB1 expression, and assessment of clinical specimens documents the expression of both PDGFRalpha and FGFR1 in rhabdoid tumor patients. PMID: 27783942
    27. The downregulation of platelet-derived growth factor receptor-alpha expression may play a causative role in imatinib-induced thrombocytopenia, a common side effect, in the subset of chronic myeloid leukemia patients with platelet-derived growth factor receptor-alpha +68 GA ins/del, +68 GA del/del, and -909C/A genotypes. PMID: 29019285
    28. These findings support a distinct contribution of PDGFRalpha signaling to hepatic stellate cell proliferation and migration. PMID: 28734947
    29. Data indicate that co-inhibition of FGFR1 and HER2 or PDGFRalpha led to enhanced drug responses. PMID: 26549034
    30. High PDGFRA expression is associated with pathogenesis of malignant peripheral nerve sheath tumor. PMID: 27477693
    31. The interaction between CSR1 and SF3A3 led to migration of SF3A3 from nucleus to cytoplasm. The cytoplasmic redistribution of SF3A3 significantly reduced the splicing efficiency of epidermal growth factor receptor and platelet-derived growth factor receptor. PMID: 27148859
    32. PDGFRa amplification in multiple skin lesions of undifferentiated pleomorphic sarcoma PMID: 28105789
    33. in addition to representing a white adipose tisseu (WAT) adipogenic niche, different PDGFRalpha(+) cell subsets modulate obesity-induced WAT fibrogenesis and are associated with loss of metabolic fitness. PMID: 28215843
    34. PDGFRA mutations were associated with gastrointestinal stromal tumors. PMID: 28098915
    35. The PDGFRA kinase domain structure we report in this study has potential to facilitate development of new agents which can inhibit this kinase, including both its activating and drug-resistant mutations. PMID: 27349873
    36. BRAF mutations are rare events in KIT/PDGFRA wild-type gastrointestinal stromal tumors. PMID: 28159677
    37. There are two platelet-derived growth factor receptor (PDGFR) genes (PDGFRA and PDGFRB), and they reside on chromosome 4 and 5. PMID: 28267575
    38. Data did not detect any significant association with SNPs of APRIL, SPATA8, PDGFRA, and POLB with Systemic Lupus Erythematosus in Chinese Han Population. PMID: 27569236
    39. Synchronous T lymphoblastic lymphoma and myeloid neoplasm with PDGFRA rearrangement. PMID: 28013529
    40. Stromal expression of PDGFRA increased with increasing histologic grade of breast phyllodes tumor. PDGFR stromal positivity was associated with shorter overall survival. PMID: 27881889
    41. genome analysis of wild-type gastrointestinal stromal tumors for mutations should include the BRAF gene in patients with KIT and PDGFRA wild-type gastrointestinal stromal tumors PMID: 27864688
    42. We demonstrate in cell lines, SCID xenografts and human tumor specimens that PDGFRalpha promotes dedifferentiation in PTC by decreasing TTF1 expression in the nucleus, which decreases iodide transport and thyroglobulin production in thyroid follicular cells. PMID: 27682510
    43. Increased PDGFRA expression is associated with thyroid papillary carcinoma. PMID: 26715280
    44. PDGFRA was a direct target of miR-34a in human pulmonary artery smooth muscle cells. PMID: 27302634
    45. Lack of PDGFRalpha(+)-cells in both the aganglionic and ganglionic Hirschsprung's disease bowel may contribute to the motility dysfunction. PMID: 27022215
    46. Ku80 and PDGFR-alpha might be effective predictive indicators for the prognosis of nasal type NK/T cell lymphoma PMID: 26778387
    47. miR-140-5p acts as a tumor suppressor during ovarian carcinogenesis, inhibiting ovarian cancer growth partially by repressing PDGFRA expression. PMID: 26297547
    48. This study identified KIT and PDGFRA mutations in 21 out of 25 gastrointestinal stromal tumor samples from 2 referential national hospitals in Peru. PMID: 25659388
    49. we retrospectively examined correlations between clinical outcomes and KIT/PDGFRA mutational status in a subset of imatinib-resistant or -intolerant patients with stromal tumor participating in a worldwide, open-label treatment-use study PMID: 26772734
    50. Data characterized metastatic exon 11 mutant gastrointestinal stromal tumors (GIST) genetic susceptibility genes beyond kit proto-oncogene protein (KIT)/PDGF alpha receptor (PDGFRalpha) genotype. PMID: 26544626

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  • 相关疾病：
    Gastrointestinal stromal tumor (GIST)
  • 亚细胞定位：
    Cell membrane; Single-pass type I membrane protein. Cell projection, cilium. Golgi apparatus.
  • 蛋白家族：
    Protein kinase superfamily, Tyr protein kinase family, CSF-1/PDGF receptor subfamily
  • 组织特异性：
    Detected in platelets (at protein level). Widely expressed. Detected in brain, fibroblasts, smooth muscle, heart, and embryo. Expressed in primary and metastatic colon tumors and in normal colon tissue.
  • 数据库链接：

    HGNC: 8803

    OMIM: 173490

    KEGG: hsa:5156

    STRING: 9606.ENSP00000257290

    UniGene: Hs.74615