FGFR3 Recombinant Monoclonal Antibody

說明書

中文名稱：

FGFR3重組抗體
貨號：

CSB-RA966627A0HU
規格：

￥1320
圖片：
Western Blot
Positive WB detected in: U87 whole cell lysate, 293 whole cell lysate, Hela whole cell lysate
All lanes: FGFR3 antibody at 1:2000
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 88, 89, 76, 86 kDa
Observed band size: 115 kDa
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品描述：

FGFR3重組單克隆抗體（產品編號：CSB-RA966627A0HU）是針對成纖維細胞生長因子受體3（FGFR3）設計的高特異性檢測工具。FGFR3作為酪氨酸激酶受體家族成員，在骨骼發育、細胞增殖及組織穩態調控中發揮重要作用，其異常表達與多種發育障礙及腫瘤發生密切相關。本產品通過重組表達技術制備，經免疫印跡（WB）和酶聯免疫吸附試驗（ELISA）嚴格驗證，在WB實驗中推薦使用1:500至1:5000的稀釋比例，可實現對內源性FGFR3蛋白的特異性識別，實驗數據表明其在多種樣本體系中均呈現清晰的單一目標條帶。該抗體適用于探索FGFR3在細胞信號轉導、組織分化等生物學過程中的分子機制研究，可支持包括蛋白表達水平分析、信號通路互作研究以及疾病相關分子標志物檢測等科研場景，為腫瘤生物學、骨骼發育異常等領域的體外實驗研究提供可靠工具。
Uniprot No.：

P22607
基因名：

FGFR3
別名：

Fibroblast growth factor receptor 3 (FGFR-3) (EC 2.7.10.1) (CD antigen CD333), FGFR3, JTK4
反應種屬：

Human
免疫原：

A synthesized peptide derived from human FGFR3
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：

6D4
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, WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
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：

Tyrosine-protein kinase that acts as cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of cell proliferation, differentiation and apoptosis. Plays an essential role in the regulation of chondrocyte differentiation, proliferation and apoptosis, and is required for normal skeleton development. Regulates both osteogenesis and postnatal bone mineralization by osteoblasts. Promotes apoptosis in chondrocytes, but can also promote cancer cell proliferation. Required for normal development of the inner ear. Phosphorylates PLCG1, CBL and FRS2. Ligand binding leads to the activation of several signaling cascades. Activation of PLCG1 leads to the production of the cellular signaling molecules diacylglycerol and inositol 1,4,5-trisphosphate. Phosphorylation of FRS2 triggers recruitment of GRB2, GAB1, PIK3R1 and SOS1, and mediates activation of RAS, MAPK1/ERK2, MAPK3/ERK1 and the MAP kinase signaling pathway, as well as of the AKT1 signaling pathway. Plays a role in the regulation of vitamin D metabolism. Mutations that lead to constitutive kinase activation or impair normal FGFR3 maturation, internalization and degradation lead to aberrant signaling. Over-expressed or constitutively activated FGFR3 promotes activation of PTPN11/SHP2, STAT1, STAT5A and STAT5B. Secreted isoform 3 retains its capacity to bind FGF1 and FGF2 and hence may interfere with FGF signaling.
基因功能參考文獻：
1. Authors found that, mechanistically, FGFR3-AS1 silencing decreased the activation of the PI3K/AKT signaling pathway. PMID: 29463348
2. Our results identified FGFR3(high)/Ki67(high) papillary pTa tumors as a subgroup with poor prognosis and encourage histological grading as high grade tumors. PMID: 30154342
3. Patients with FGFR3 mutations or FGFR3-TACC3 fusion may constitute potential candidates for a novel FGFR-targeted therapy in the perioperative setting. PMID: 30064409
4. Data suggest that FGFR3 with mutation found in SADDAN (but not FGFR3 with mutation found in TDII) affects cytoskeleton organization in chondrocytes by inducing tyrosine hyperphosphorylation of paxillin; binding of FGFR3 to PLCG1 appears to be involved. (PLCG1 = phospholipase C gamma 1; SADDAN = Severe Achondroplasia with Developmental Delay and Acanthosis Nigricans; TDII = Thanatophoric Dysplasia type II) PMID: 29242050
5. Here, we present a case with prenatal ultrasonographic findings suggestive of TD, and highlight the patient's postnatal dysmorphic features and typical radiographic findings. The definitive diagnosis of TD type I (TDI) was made postnatally, when molecular genetic analysis revealed the previously described p.R248C mutation in FGFR3. This case is reported due to its relative long life span and the molecular diagnosis. PMID: 30226972
6. FGFR3 expression indicated an adverse prognosis for lung adenocarcinoma individuals and promoted metastatic potential of lung adenocarcinoma cells PMID: 29850625
7. FGFR1, as well as its downstream regulatory PI3K/AKT kinases, may serve as potential biomarkers for the invasiveness and prognosis of laryngeal cancer. PMID: 29299828
8. FGFR3-AS1 expression levels were higher in high grade tumors than those in low grade tumors. FGFR3-AS1 expression levels were higher in invasive tumors than those in non-invasive bladder tumors. PMID: 29226855
9. Disease-free survival (DFS) was then calculated according to FGFR3 IHC expression. PMID: 30061236
10. The gene FGFR3 is responsible for the production of the FGFR 3 protein that converts cartilage to bone. All people with a single copy of the mutated gene FGFR3 have Achondroplasia. PMID: 29185944
11. genetic association studies in pediatric population in Japan: Data suggest that mutations in ACAN (aggrecan), FGFR3 (fibroblast growth factor receptor-3), or GHRHR (growth- hormone-releasing-hormone receptor) are associated with idiopathic short stature in the population studied. PMID: 28768959
12. HPV-positive vulvar squamous cell carcinoma is characterized by oncogenic FGFR3 mutations that helps classify this subtype as a separate disease. Inhibitors of FGFR3 merit consideration as a therapeutic strategy in this neglected cancer in women PMID: 28377483
13. Results show that olfactory neuroblastoma tumors harbor recurrent chromosomal copy-number changes, including FGFR3 amplification associated with overexpression. PMID: 28775129
14. FGFR3-TACC3 is a recurrent resistance mechanism, which can bypass EGFR blockade by all generations of EGFR TKIs in NSCLC. PMID: 28838400
15. Mutation in the FGFR3 gene is associated with with Klinefelter syndrome and achondroplasia. PMID: 28672740
16. Genetic screening of the family revealed a previously reported heterozygous c.1138 G > A (p.G380R) mutation in the coding exon 8 of FGFR3 gene PMID: 28679403
17. FGFR3 mutations have very limited urothelial tumorigenicity and that these mutations must collaborate with other genetic events to drive urothelial tumorigenesis. PMID: 27157475
18. Long-term dovitinib administration was not feasible due to frequent toxicity. Absent clinical activity suggests that patient selection by pFGFR3 IHC alone does not enrich for response to FGFR3 kinase inhibitors in urothelial carcinoma. PMID: 27932416
19. Results provide evidence that FGFR3 mutations in human papillomavirus positive tonsillar and base of tongue cancer is indicative of worse prognosis. PMID: 28525363
20. Increased levels of FGFR3 and PIK3CA mutated DNA in urine and plasma are indicative of later progression and metastasis in bladder cancer. PMID: 28069289
21. FGFR3 expression increased in classical and neural subtypes of glioma and was associated with differentiated cellular functions. FGFR3 showed very limited correlation with other common receptor tyrosine kinases, and predicted improved survival for glioma patients. PMID: 27829236
22. REVIEW. FGFR3 is expressed in chondrocytes and mature osteoblasts where it functions to regulate bone growth. Analysis of the mutations in FGFR3 revealed increased signaling through a combination of mechanisms PMID: 27987249
23. There was a lower frequency of mutation in FGFR3, a gene associated with low-risk Bladder Cancer, than reported in the The Cancer Genome Atlas database. PMID: 27520487
24. A higher expression of FGFR3, phosphorylated AKT, and ZEB1 were observed. PMID: 27267856
25. FGFR3 mutation status seems promising to guide decision-making on adjuvant anti-FGFR3 therapy as it appeared homogeneous in RC and lymph nodes +. Based on the results of TUR, the deep part of the tumor needs to be assessed if neoadjuvant anti-FGFR3 treatment is considered PMID: 27091807
26. We present the first actionable mutation spectrum in Indian lung cancer genome. These findings implicate FGFR3 as a novel therapeutic in lung adenocarcinoma. PMID: 27998968
27. FGFR2, TWIST1, and FGFR3 mutations were identified in children with tracheal cartilaginous sleeve (TCS). All five children with a W290C mutation in FGFR2 had TCS, and most previously reported children with W290C had identification of TCS or early death PMID: 27228464
28. The Gly380Arg and Asn540Lys are hot spot mutations of the FGFR3 gene among patients with ACH/HCH. PMID: 28777845
29. the FGFR3 gene is an infrequent target in the pathogenesis of Han Chinese urothelial cell carcinoma PMID: 27029078
30. Our results extend the genetic mutation spectrum of FGFR3. PMID: 29080836
31. Study found FGFR3 gene mutation plus GRB10 gene duplication in a patient with achondroplasia plus growth delay with prenatal onset PMID: 27370225
32. Our family confirms the consistent and unique phenotype of this condition, and the specificity of the mutation in FGFR3. PMID: 27139183
33. no insertions or deletions in FGFR3 have been reported to cause thanatophoric dysplasia types 1 or 2; therefore, this represents the first report to describe such a mutation. PMID: 27028100
34. results suggest that FGFR3 kinase activity may regulate the papillomavirus reproductive program through phosphorylation of the E2 protein although this is unlikely to occur through the Y102 residue of HPV E2. PMID: 28768864
35. Our data also reinforce the notion that molecular testing of FGFR3 must be included in the diagnostic approach of coronal craniosynostosis. This will allow accurate genetic counseling and optimal management of MS, which might otherwise go undiagnosed because of mild manifestations and wide variability of expression PMID: 27568649
36. We describe the first case of protein-losing enteropathy in a pediatric patient, with severe skeletal dysplasia consistent with thanatophoric dysplasia type I and DNA analysis that revealed a c.1949A>T (p.Lys650Met) in exon 15 of the FGFR3 gene. PMID: 27214123
37. High FGFR3 expression is associated with bladder cancer. PMID: 28320388
38. Study provides evidence of the significant oncogenic potential of the FGFR3-TACC3 fusion protein. The presence of the TACC coiled-coil domain leads to increased and altered levels of FGFR3 activation, fusion protein phosphorylation, downstream signaling, cellular transformation, proliferation, and viability. PMID: 26869289
39. we show that low dose of NVP-BGJ398 improves in vivo condyle growth and corrects dysmorphologies in Fgfr3(Y367C/+) mice, suggesting that postnatal treatment with NVP-BGJ398 mice might offer a new therapeutic strategy to improve mandible anomalies in achondroplasia (ACH), and others FGFR3-related disorders. PMID: 27260401
40. FGFR3 mRNA missplicing in hepatocellular carcinoma (HCC), contributes significantly to its malignant character. PMID: 27267910
41. Mutations in FGFR3 gene is associated with tubular adenomas. PMID: 27438523
42. High FGFR3 expression is associated with multiple myeloma. PMID: 27509849
43. FGFR3 was predominantly mutated in infiltrative hepatocellular carcinoma (HCC) compared to nodular HCC. FGFR3 protein expression was higher in mutated infiltrative HCC compared to non-mutated infiltrative HCC and nodular HCC. FGFR3 may be a candidate oncogene in tumor progression. PMID: 28058595
44. Our findings show that grade heterogeneity in urothelial carcinoma is characterized by increased MIB-1 labelling, and particularly in the FGFR3 mutant pathway, with homozygous deletions of CDKN2A in low- and high-grade areas PMID: 27530957
45. We argue that routine use of molecular genomic tumour analysis in urothelial carcinoma may inform selection of patients for appropriate trials and we further investigate the potential of FGFR3 as a meaningful clinical target for this difficult disease PMID: 27271022
46. In conclusion, we identified four novel loci (TGFA, PIK3R1, FGFR3 and TREH) and confirmed two loci known to be associated with cartilage thickness.The identified associations were not caused by rare exonic variants. This is the first report linking TGFA to human Osteoarthritis, which may serve as a new target for future therapies. PMID: 27701424
47. Case Report: FGFR3 epidermal naevus syndrome with urothelial mosaicism for activating p.Ser249Cys FGFR3 mutation. PMID: 27786351
48. FGFR alterations are not frequent in low-grade gliomas, they are more common in hemispheric low-grade gliomas and are important since targeted therapies exist for FGFR receptors. PMID: 27659822
49. FGFR3 gene mutations are associated with Urinary Bladder Cancer. PMID: 27356691
50. we identified a novel FGFR3 mutation, p.Ser348Cys, in a patient with achondroplasia. A number of different FGFR3 mutations can cause achondroplasia; therefore, if the common p.Gly380Arg mutation is not found, complete analysis of FGFR3 is indicated in patients with achondroplasia PMID: 26754866
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相關疾病：

Achondroplasia (ACH); Crouzon syndrome with acanthosis nigricans (CAN); Thanatophoric dysplasia 1 (TD1); Thanatophoric dysplasia 2 (TD2); Hypochondroplasia (HCH); Bladder cancer (BLC); Cervical cancer (CERCA); Camptodactyly, tall stature, and hearing loss syndrome (CATSHLS); Multiple myeloma (MM); Lacrimo-auriculo-dento-digital syndrome (LADDS); Keratinocytic non-epidermolytic nevus (KNEN); Muenke syndrome (MNKS); Keratosis, seborrheic (KERSEB); Testicular germ cell tumor (TGCT); Achondroplasia, severe, with developmental delay and acanthosis nigricans (SADDAN)
亞細胞定位：

[Isoform 1]: Cell membrane; Single-pass type I membrane protein. Cytoplasmic vesicle. Endoplasmic reticulum. Note=The activated receptor is rapidly internalized and degraded. Detected in intracellular vesicles after internalization of the autophosphorylated receptor.; [Isoform 2]: Cell membrane; Single-pass type I membrane protein.; [Isoform 3]: Secreted.; [Isoform 4]: Cell membrane; Single-pass type I membrane protein.
蛋白家族：

Protein kinase superfamily, Tyr protein kinase family, Fibroblast growth factor receptor subfamily
組織特異性：

Expressed in brain, kidney and testis. Very low or no expression in spleen, heart, and muscle. In 20- to 22-week old fetuses it is expressed at high level in kidney, lung, small intestine and brain, and to a lower degree in spleen, liver, and muscle. Isof
數據庫鏈接：

HGNC: 3690

OMIM: 100800

KEGG: hsa:2261

STRING: 9606.ENSP00000339824

UniGene: Hs.1420

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