PDGFRA (tovetumab Biosimilar) Recombinant Monoclonal Antibody

說明書

貨號：

CSB-RA017712MB1HU
規格：
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P16234
基因名：

PDGFRA
別名：

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
反應種屬：

Human
免疫原：

Recombinant Human PDGFRA protein
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
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 7.173
產品提供形式：

Liquid
應用說明：

Validation Status
Application-specific performance (e.g., in flow cytometry, ELISA, IHC or other assay formats) has not yet been experimentally verified by CUSABIO. Users are advised to determine the optimal working conditions empirically in their own assay systems.
Guaranteed Quality
① Antibody purity?> 95% tested by SDS-PAGE.
② Endotoxin level < 0.1EU/ug tested by LAL method.
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

In stock
用途：

It is a non-therapeutic biosimilar antibody, owning the same variable region from the corresponding approved therapeutic antibody. In conclusion, it is a research-grade biosimilar antibody and expressed in mammalian cell, which can be directly used as positive controls in drug discovery or used for rapid verification of the biological functions of target protein.

產品評價

靶點詳情

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.
基因功能參考文獻：
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 authors 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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相關疾病：

Gastrointestinal stromal tumor (GIST)
亞細胞定位：

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
組織特異性：

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.
數據庫鏈接：

HGNC: 8803

OMIM: 173490

KEGG: hsa:5156

STRING: 9606.ENSP00000257290

UniGene: Hs.74615

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