磷酸化碱性成纤维细胞生长因子受体1抗体

相关标记 Alexa Fluor 350 Alexa Fluor 488 Alexa Fluor 555 Alexa Fluor 647 AP APC Biotin Cy3 Cy5

Cy5.5 Cy7 FITC Gold HRP PE PE-Cy3 PE-CY5 PE-CY5.5 PE-CY7 RBITC

英文名称Phospho-FGFR1+FGFR2 (Tyr463)

中文名称磷酸化碱性成纤维细胞生长因子受体1抗体

别 名bFGF R; BFGFR; C FGR; CD 331; CD331; CD331 antigen; CEK; FGFBR; FGFR 1; Fibroblast growth factor receptor 1; FLG; FLG protein; FLJ14326; FLT 2; FLT2; Fms like tyrosine kinase 2; Fms related tyrosine kinase 2; Fms related tyrosine kinase 2 Pfeiffer syndrome; H2; H3; H4; H5; HBGFR; Heparin binding growth factor receptor; Hydroxyaryl protein kinase; KAL 2; KAL2; MFR; N SAM; N sam tyrosine kinase; Protein tyrosine kinase; Tyrosylprotein kinase; Basic fibroblast growth factor receptor 1.

磷酸化碱性成纤维细胞生长因子受体1抗体产品类型磷酸化抗体

研究领域肿瘤 免疫学 生长因子和激素 转录调节因子

抗体来源Rabbit

克隆类型Polyclonal

交叉反应Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit,

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 ICC=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.

分 子 量88kDa

磷酸化碱性成纤维细胞生长因子受体1抗体细胞定位细胞膜

性 状Lyophilized or Liquid

浓 度1mg/1ml

免 疫 原KLH conjugated Synthesised phosphopeptide derived from human FGFR1/FGFR2 around the phosphorylation site of Tyr463

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

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.

磷酸化碱性成纤维细胞生长因子受体1抗体产品介绍background:

Fibroblast growth factors (FGFs) produce mitogenic ａnd angiogenic effects in target cells by signaling through the cellular surface tyrosine kinase receptors. There are four members of the FGF receptor family: FGFR-1 (flg), FGFR-2 (bek, KGFR), FGFR-3 ａnd FGFR-4. Each receptor contains an extracellular ligand binding domain, a transmembrane region ａnd a cytoplasmic kinase domain (1). Following ligand binding ａnd dimerization, the receptors are phosphorylated at specific tyrosine residues (2). Seven tyrosine residues in the cytoplasmic tail of FGFR-1 can be phosphorylated: Tyr463, Tyr583, Tyr585, Tyr653, Tyr654, Tyr730 ａnd Tyr766. Tyrosine 653 ａnd 654 are important for catalytic activity of the activated FGFR ａnd are essential for signaling (3). The other phosphorylated tyrosine residues may provide docking sites for downstream signaling components such as Crk ａnd PLCgamma.

Function:

Receptor for fibroblast growth factors FGF2 ａnd FGF1. Receptor for FGF23 in the presence of KL (By similarity). Promotes mitogenesis in response to fibroblast growth factors. Activates PLCG1.

Subunit:

Homodimer. Interacts with KLB. Interacts with KL ａnd FGF23. Interacts with SHB ａnd GRB10. Interacts with PLCG1 (via SH2 domains). Interacts with KAL1; this interaction does not interfere with FGF2-binding to FGFR1, but prevents binding of heparin-bound FGF2. Interacts with SOX2 ａnd SOX3.

Subcellular Location:

Membrane; Single-pass type I membrane protein.

Tissue Specificity:

Detected in astrocytoma, neuroblastoma ａnd adrenal cortex cell lines. Some isoforms are detected in foreskin fibroblast cell lines, however isoform 17, isoform 18 ａnd isoform 19 are not detected in these cells.

Post-translational modifications:

Binding of FGF1 ａnd heparin promotes autophosphorylation on tyrosine residues ａnd activation of the receptor.

DISEASE:

Defects in FGFR1 are a cause of Pfeiffer syndrome (PS) [MIM:101600]; also known as acrocephalosyndactyly type V (ACS5). PS is characterized by craniosynostosis (premature fusion of the skull sutures) with deviation ａnd enlargement of the thumbs ａnd great toes, brachymesophalangy, with phalangeal ankylosis ａnd a varying degree of soft tissue syndactyly.

Defects in FGFR1 are a cause of idiopathic hypogonadotropic hypogonadism (IHH) [MIM:146110]. IHH is defined as a deficiency of the pituitary secretion of follicle-stimulating hormone ａnd luteinizing hormone, which results in the impairment of pubertal maturation ａnd of reproductive function.

Important Note:

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

Defects in FGFR1 are the cause of Kallmann syndrome type 2 (KAL2) [MIM:147950]; also known as hypogonadotropic hypogonadism ａnd anosmia. Anosmia or hyposmia is related to the absence or hypoplasia of the olfactory bulbs ａnd tracts. Hypogonadism is due to deficiency in gonadotropin-releasing hormone ａnd probably results from a failure of embryonic migration of gonadotropin-releasing hormone-synthesizing neurons. In some cases, midline cranial anomalies (cleft lip/palate ａnd imperfect fusion) are present ａnd anosmia may be absent or inconspicuous.

Defects in FGFR1 are the cause of osteoglophonic dysplasia (OGD) [MIM:166250]; also known as osteoglophonic dwarfism. OGD is characterized by craniosynostosis, prominent supraorbital ridge, ａnd depressed nasal bridge, as well as by rhizomelic dwarfism ａnd nonossifying bone lesions. Inheritance is autosomal dominant.

Defects in FGFR1 are the cause of trigonocephaly non-syndromic (TRICEPH) [MIM:190440]; also known as metopic craniosynostosis. The term trigonocephaly describes the typical keel-shaped deformation of the forehead resulting from premature fusion of the frontal suture. Trigonocephaly may occur also as a part of a syndrome.

Note=A chromosomal aberration involving FGFR1 may be a cause of stem cell leukemia lymphoma syndrome (SCLL). Translocation t(8;13)(p11;q12) with ZMYM2. SCLL usually presents as lymphoblastic lymphoma in association with a myeloproliferative disorder, often accompanied by pronounced peripheral eosinophilia and/or prominent eosinophilic infiltrates in the affected bone marrow.

[DISEASE] Note=A chromosomal aberration involving FGFR1 may be a cause of stem cell myeloproliferative disorder (MPD). Translocation t(6;8)(q27;p11) with FGFR1OP. Insertion ins(12;8)(p11;p11p22) with FGFR1OP2. MPD is characterized by myeloid hyperplasia, eosinophilia ａnd T-cell or B-cell lymphoblastic lymphoma. In general it progresses to acute myeloid leukemia. The fusion proteins FGFR1OP2-FGFR1, FGFR1OP-FGFR1 or FGFR1-FGFR1OP may exhibit constitutive kinase activity ａnd be responsible for the transforming activity.

Note=A chromosomal aberration involving FGFR1 may be a cause of stem cell myeloproliferative disorder (MPD). Translocation t(8;9)(p12;q33) with CEP110. MPD is characterized by myeloid hyperplasia, eosinophilia ａnd T-cell or B-cell lymphoblastic lymphoma. In general it progresses to acute myeloid leukemia. The fusion protein CEP110-FGFR1 is found in the cytoplasm, exhibits constitutive kinase activity ａnd may be responsible for the transforming activity.

碱性成纤维细胞生长因子(bFGF)是一种多功能的生长因子,具有促进细胞有丝分裂和诱导新血管形成作用,和其受体(FGFR1)碱性成纤维细胞生长因子受体结合之后,在人体各组织中具有广泛的生物学活性和生理病理作用.越来越多的研究表明肿瘤细胞中bFGF-R1过度表达对肿瘤发生发展具有重要意义。ermined by the end user.