Recombinant Human Fibroblast growth factor receptor 3 (FGFR3), partial (Active)

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品名簡(jiǎn)稱：

Recombinant Human FGFR3 protein, partial (Active)
貨號(hào)：

CSB-MP008646HU
說明書：

MSDS Datasheet
規(guī)格：

￥684
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

Activity
Measured by its binding ability in a functional ELISA. Immobilized Human FGFR3 at 2 μg/ml can bind Anti-FGFR3 recombinant antibody (CSB-RA008646MA1HU). The EC₅₀ is 0.6767-0.8946 ng/mL. Biological Activity Assay

The purity of FGFR3 was greater than 95% as determined by SEC-HPLC
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

Greater than 90% as determined by SDS-PAGE.
Greater than 95% as determined by SEC-HPLC.
內(nèi)毒素：

Less than 1.0 EU/ug as determined by LAL method.
生物活性：

Measured by its binding ability in a functional ELISA. Immobilized Human FGFR3 at 2 μg/mL can bind Anti-FGFR3 recombinant antibody (CSB-RA008646MA1HU). The EC50 is 0.6767-0.8946 ng/mL.
基因名：

FGFR3
Uniprot No.：

P22607
別名：

Fibroblast growth factor receptor 3; EC:2.7.10.1; FGFR-3; CD333; FGFR3; JTK4
種屬：

Homo sapiens (Human)
蛋白長(zhǎng)度：

Partial
來源：

Mammalian cell
分子量：

39.5 kDa
表達(dá)區(qū)域：

23-375aa
氨基酸序列

ESLGTEQRVVGRAAEVPGPEPGQQEQLVFGSGDAVELSCPPPGGGPMGPTVWVKDGTGLVPSERVLVGPQRLQVLNASHEDSGAYSCRQRLTQRVLCHFSVRVTDAPSSGDDEDGEDEAEDTGVDTGAPYWTRPERMDKKLLAVPAANTVRFRCPAAGNPTPSISWLKNGREFRGEHRIGGIKLRHQQWSLVMESVVPSDRGNYTCVVENKFGSIRQTYTLDVLERSPHRPILQAGLPANQTAVLGSDVEFHCKVYSDAQPHIQWLKHVEVNGSKVGPDGTPYVTVLKTAGANTTDKELEVLSLHNVTFEDAGEYTCLAGNSIGFSHHSAWLVVLPAEEELVEADEAGSVYAG
蛋白標(biāo)簽：

C-terminal 10xHis-tagged
產(chǎn)品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
緩沖液：

Lyophilized from a 0.2 μm sterile filtered PBS, 6% Trehalose, pH 7.4
復(fù)溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲(chǔ)存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質(zhì)期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Basically, we can dispatch the products out in 3-7 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
注意事項(xiàng)：

Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.
Datasheet & COA：

Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)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(xiàn)：
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(guān)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(xì)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(shù)據(jù)庫鏈接：

HGNC: 3690

OMIM: 100800

KEGG: hsa:2261

STRING: 9606.ENSP00000339824

UniGene: Hs.1420