MyBioSource

antibody

MAb to FGF Receptor 1 alpha

MBS312068

0.25 mg

695 USD

monoclonal

mouse

MBS312068

Antibody

MAb to FGF Receptor 1 alpha

Fibroblast Growth Factor Receptor 1 alpha

Monoclonal Antibody to Human Fibroblast Growth Factor Receptor 1 alpha (FGFr1a)

basic fibroblast growth factor receptor 1 isoform 1; Basic fibroblast growth factor receptor 1; basic fibroblast growth factor receptor 1; OTTHUMP00000190874; OTTHUMP00000190875; OTTHUMP00000190876; OTTHUMP00000190877; OTTHUMP00000190879; OTTHUMP00000190880; OTTHUMP00000190881; OTTHUMP00000190885; proto-oncogene c-Fgr; FMS-like tyrosine kinase 2; hydroxyaryl-protein kinase; fms-related tyrosine kinase 2; heparin-binding growth factor receptor; fibroblast growth factor receptor 1; Fms-like tyrosine kinase 2; FLT-2; Proto-oncogene c-Fgr

FGFR1; FGFR1; CEK; FLG; OGD; FLT2; KAL2; BFGFR; CD331; FGFBR; FLT-2; HBGFR; N-SAM; FGFR-1; FLJ99988; bFGF-R-1; FGFBR; FLG; FLT2

FGFR1_HUMAN

Monoclonal

IgG2a

M2F12

Mouse

822

Fibroblast Growth Factor Receptor 1 alpha. Reacts with the NH2-terminus of unique NH2-terminal Ig loop of FGFr1. Epitope is within the sequence between glu30 and ala74 of FGFr1a. Reacts with human, rat and mouse receptors.

Protein G chromatography

Purified, Liquid

0.7mg/ml (OD280nm)

Aliquot and store (up to 1 year) at -20 degree C to -70 degree C. Avoid multiple freeze/thaw cycles.

Immunogen: Recombinant human ectodomain of FGFr1a expressed in E. coli beginning with pro23, antigen contained NH2-terminal gly-ser-pro-gly-ile and COOH-terminal glu-phe sequences. Affinity Constant: Not determined

Buffer: PBS, pH 7.4. Preservative: No. Important Note: Centrifuge before opening to ensure complete recovery of vial contents.

Monoclonal Antibodies to Cytokines and Growth Factors

Xu, et al., (1992), J. Biol. Chem., 267:17792. Proudovsky, et al., (1994), J. Biol. Chem., 269:31720. Morrison, et al., (1994) Cancer Res., 54:2794. Zhao, et al., (1994), J. Clin. Invest., 94:992.<b

105990522

NP_075598.2

NM_023110.2

P11362

91,868 Da

Adherens Junction Pathway 83070!!Adherens Junction Pathway 481!!Axon Guidance Pathway 105688!!Downstream Signaling Of Activated FGFR Pathway 160957!!Endochondral Ossification Pathway 198812!!FGF Signaling Pathway 137989!!FGFR Ligand Binding And Activation Pathway 106344!!FGFR1 Ligand Binding And Activation Pathway 106345!!FGFR1c Ligand Binding And Activation Pathway 106347!!FRS2-mediated Cascade Pathway 160958

The protein encoded by this gene is a member of the fibroblast growth factor receptor (FGFR) family, where amino acid sequence is highly conserved between members and throughout evolution. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein consists of an extracellular region, composed of three immunoglobulin-like domains, a single hydrophobic membrane-spanning segment and a cytoplasmic tyrosine kinase domain. The extracellular portion of the protein interacts with fibroblast growth factors, setting in motion a cascade of downstream signals, ultimately influencing mitogenesis and differentiation. This particular family member binds both acidic and basic fibroblast growth factors and is involved in limb induction. Mutations in this gene have been associated with Pfeiffer syndrome, Jackson-Weiss syndrome, Antley-Bixler syndrome, osteoglophonic dysplasia, and autosomal dominant Kallmann syndrome 2. Chromosomal aberrations involving this gene are associated with stem cell myeloproliferative disorder and stem cell leukemia lymphoma syndrome. Alternatively spliced variants which encode different protein isoforms have been described; however, not all variants have been fully characterized. [provided by RefSeq]

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. Ref.20 Ref.35 Ref.38 Ref.39. Catalytic activity: ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. Ref.20 Ref.38 Ref.39. Subunit structure: Homodimer. Interacts with KLB. By similarity. Interacts with KL and FGF23. By similarity. 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. By similarity. Ref.20 Ref.22 Ref.24 Ref.26 Ref.32 Ref.38 Ref.39. 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. Ref.18. Post-translational modification: Binding of FGF1 and heparin promotes autophosphorylation on tyrosine residues and activation of the receptor. Involvement in 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. Ref.40Defects 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. Ref.48 Ref.51Defects 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. Ref.42 Ref.43 Ref.45 Ref.46 Ref.48 Ref.49 Ref.50 Ref.52Defects 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. Ref.44 Ref.47Defects 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. Ref.41Note=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.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. Sequence similarities: 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.

0.25 mg

695 USD