成纤维细胞生长因子受体2抗体

成纤维细胞生长因子受体2抗体英文名称  Anti-FGFR2/KGFR/CD332 

中文名称  成纤维细胞生长因子受体2抗体 

别    名  KGFR; KSAM; Bacteria expressed kinase; BEK; BEK fibroblast growth factor receptor; BFR 1; BFR1; CD 332; CD332; CD332 antigen; CEK 3; CEK3; CFD 1; CFD1; Craniofacial dysostosis 1; Crouzon syndrome; ECT 1; ECT 1; ECT1; FGF receptor; FGFR 2; FGFR-2; Fgfr2; FGFR2_HUMAN; Fibroblast growth factor receptor 2; Hydroxyaryl protein kinase; Hydroxyaryl protein kinase; Jackson Weiss syndrome; JWS; JWS antibody K SAM; K sam protein; K sam protein; K-sam ; Keratinocyte growth factor receptor 2; Keratinocyte growth factor receptor; Pfeiffer syndrome; Protein tyrosine kinase receptor like 14; TK14; TK25; Tyrosylprotein kinase; Tyrosylprotein kinase. 

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg                                  

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat  

产品类型  一抗    

研究领域  肿瘤 心血管 免疫学 神经生物学 信号转导 干细胞 生长因子和激素 激酶和磷酸酶  

蛋白分子量  predicted molecular weight: 89kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human FGFR2 N-terminus 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500 

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

成纤维细胞生长因子受体2抗体产品介绍 Fibroblast growth factors (FGFs) are members of a large family of structurally related polypeptides that are potent physiological regulators of growth and differentiation for a wide variety of cells of mesodermal, ectodermal and endodermal origin. Four genes encoding for high affinity cell surface FGF receptors (FGFRs) have been identified: FGFR1, FGFR2, FGFR3 and FGFR4. FGFRs are members of the tyrosine kinase family of growth factor receptors. FGFR2 is highly expressed in developing human tissues including the brain, choroids plexus, lung etc. Alternative names: Bacteria expressed kinase; BEK; BFR 1; BFR1; CD 332; CD332; CD332 antigen; CEK 3; CEK3; CFD 1; CFD1; Craniofacial dysostosis 1; Crouzon syndrome; ECT 1; ECT1; FGFR 2; Fibroblast growth factor receptor 2; Hydroxyaryl protein kinase; Jackson Weiss syndrome; JWS; K SAM; K sam protein; Keratinocyte growth factor receptor 2; Keratinocyte growth factor receptor; KGFR; KSAM; Pfeiffer syndrome; Protein tyrosine kinase receptor like 14; TK14; TK25; Tyrosylprotein kinase.

Function : 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, migration and apoptosis, and in the regulation of embryonic development. Required for normal embryonic patterning, trophoblast function, limb bud development, lung morphogenesis, osteogenesis and skin development. Plays an essential role in the regulation of osteoblast differentiation, proliferation and apoptosis, and is required for normal skeleton development. Promotes cell proliferation in keratinocytes and immature osteoblasts, but promotes apoptosis in differentiated osteoblasts. Phosphorylates PLCG1, FRS2 and PAK4. 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. FGFR2 signaling is down-regulated by ubiquitination, internalization and degradation. Mutations that lead to constitutive kinase activation or impair normal FGFR2 maturation, internalization and degradation lead to aberrant signaling. Over-expressed FGFR2 promotes activation of STAT1.

Subunit : Monomer. Homodimer after ligand binding. Interacts predominantly with FGF1 and FGF2, but can also interact with FGF3, FGF4, FGF6, FGF7, FGF8, FGF9, FGF10, FGF17, FGF18 and FGF22 (in vitro). Ligand specificity is determined by tissue-specific expression of isoforms, and differences in the third Ig-like domain are crucial for ligand specificity. Isoform 1 has high affinity for FGF1 and FGF2, but low affinity for FGF7. Isoform 3 has high affinity for FGF1 and FGF7, and has much higher affinity for FGF7 than isoform 1 (in vitro). Affinity for fibroblast growth factors (FGFs) is increased by heparan sulfate glycosaminoglycans that function as coreceptors. Likewise, KLB increases the affinity for FGF19 and FGF21. Interacts with PLCG1, GRB2 and PAK4.

Subcellular Location : Cell membrane; Single-pass type I membrane protein. Golgi apparatus. Cytoplasmic vesicle. Note=Detected on osteoblast plasma membrane lipid rafts. After ligand binding, the activated receptor is rapidly internalized and degraded.

Isoform 1: Cell membrane; Single-pass type I membrane protein. Note=After ligand binding, the activated receptor is rapidly internalized and degraded.

Isoform 3: Cell membrane; Single-pass type I membrane protein. Note=After ligand binding, the activated receptor is rapidly internalized and degraded.

Post-translational modifications : N-glycosylated in the endoplasmic reticulum. The N-glycan chains undergo further maturation to an Endo H-resistant form in the Golgi apparatus. 

Ubiquitinated. FGFR2 is rapidly ubiquitinated after autophosphorylation, leading to internalization and degradation. Subject to degradation both in lysosomes and by the proteasome.

DISEASE : Defects in FGFR2 are the cause of Crouzon syndrome (CS) ; also called craniofacial dysostosis type I (CFD1). CS is an autosomal dominant syndrome characterized by craniosynostosis (premature fusion of the skull sutures), hypertelorism, exophthalmos and external strabismus, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism. Defects in FGFR2 are a cause of Jackson-Weiss syndrome (JWS) [MIM:123150]. JWS is an autosomal dominant craniosynostosis syndrome characterized by craniofacial abnormalities and abnormality of the feet: broad great toes with medial deviation and tarsal-metatarsal coalescence. 

Similarity : Belongs to the protein kinase superfamily. Tyr protein kinase family. Fibroblast growth factor receptor subfamily. Contains 3 Ig-like C2-type (immunoglobulin-like) domains. Contains 1 protein kinase domain.

Database links : UniProtKB/Swiss-Prot: P21802.1 

KGFR又称FGFR2（Fibroblast Growth Factor Receptor 2）成纤维细胞生长因子受体又称：纤维母细胞生长因子受体2，是FGFRs家族的一员。不同的FGFR对FGF亲和力不同，在组织的分布也不一样。FGFR-2对细胞的增殖、分化、血管生成、胚胎及骨骼发育和在与生长发育相关的进程中起着十分重要的作用.