FGFR-2, Monoclonal Antibody

FGFR2; BEK; JWS; CEK3; CFD1; ECT1; KGFR; TK14; TK25; BFR-1; CD332; K-SAM

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 10; NC_000010.11 (121478330..121598458, complement). Location: 10q26

Monoclonal

IgG1

(6L19)

Mouse

Lyophilized

Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-FGFR-2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Fibroblast Growth Factor Receptor 2 (FGF R2) is one of four closely related transmembrane tyrosine kinases (FGF R1-4) that function as receptors for the fibroblast growth factor family. Multiple isoforms are generated by alternative mRNA splicing resulting in extracellular domains with three (alpha isoforms) or two (beta isoforms) Ig-like domains. In addition, alternative exon usage in the Ig III (membrane proximal) domain results in IIIb or IIIc isoforms.

Western Blot (WB), Immunohistochemistry (IHC) - Paraffin, Neutr

92,025 Da

fibroblast growth factor receptor 2

fibroblast growth factor receptor 2; FGFR-2; FGF receptor; soluble FGFR4 variant 4; bacteria-expressed kinase; hydroxyaryl-protein kinase; FGFR2-AHCYL1 fusion kinase protein; keratinocyte growth factor receptor; BEK fibroblast growth factor receptor; protein tyrosine kinase, receptor like 14

Fibroblast growth factor receptor 2

BEK; KGFR; KSAM; FGFR-2; KGFR  [Similar Products]

FGFR2_HUMAN

The protein encoded by this gene is a member of the fibroblast growth factor receptor 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 is a high-affinity receptor for acidic, basic and/or keratinocyte growth factor, depending on the isoform. Mutations in this gene are associated with Crouzon syndrome, Pfeiffer syndrome, Craniosynostosis, Apert syndrome, Jackson-Weiss syndrome, Beare-Stevenson cutis gyrata syndrome, Saethre-Chotzen syndrome, and syndromic craniosynostosis. Multiple alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jan 2009]

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. Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35 Ref.36 Ref.38 Ref.40 Ref.47

Catalytic activity: ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. Ref.31 Ref.35 Ref.49 Ref.51

Enzyme regulation: Present in an inactive conformation in the absence of bound ligand. Ligand binding leads to dimerization and activation by autophosphorylation on tyrosine residues. Inhibited by ARQ 523 and ARQ 069; these compounds maintain the kinase in an inactive conformation and inhibit autophosphorylation. Ref.48 Ref.51

Subunit structure: 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. Ref.6 Ref.7 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.36 Ref.43 Ref.47 Ref.49

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. Ref.31 Ref.33 Ref.34 Ref.36Isoform 1: Cell membrane; Single-pass type I membrane protein. Note: After ligand binding, the activated receptor is rapidly internalized and degraded. Ref.31 Ref.33 Ref.34 Ref.36Isoform 3: Cell membrane; Single-pass type I membrane protein. Note: After ligand binding, the activated receptor is rapidly internalized and degraded. Ref.31 Ref.33 Ref.34 Ref.36Isoform 14: Secreted Ref.31 Ref.33 Ref.34 Ref.36. Isoform 19: Secreted Ref.31 Ref.33 Ref.34 Ref.36.

Domain: The second and third Ig-like domains directly interact with fibroblast growth factors (FGF) and heparan sulfate proteoglycans. Alternative splicing events affecting the third Ig-like domain are crucial for ligand selectivity. Ref.6 Ref.7 Ref.25

Post-translational modification: Autophosphorylated. Binding of FGF family members together with heparan sulfate proteoglycan or heparin promotes receptor dimerization and autophosphorylation on several tyrosine residues. Autophosphorylation occurs in trans between the two FGFR molecules present in the dimer. Phosphorylation at Tyr-769 is essential for interaction with PLCG1. Ref.29 Ref.31 Ref.33 Ref.35 Ref.48 Ref.49 Ref.50 Ref.51N-glycosylated in the endoplasmic reticulum. The N-glycan chains undergo further maturation to an Endo H-resistant form in the Golgi apparatus. Ref.31 Ref.33Ubiquitinated. FGFR2 is rapidly ubiquitinated after autophosphorylation, leading to internalization and degradation. Subject to degradation both in lysosomes and by the proteasome. Ref.28 Ref.31 Ref.38

Involvement in disease: Crouzon syndrome (CS) [MIM:123500]: An autosomal dominant syndrome characterized by craniosynostosis, hypertelorism, exophthalmos and external strabismus, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10 Ref.24 Ref.40 Ref.48 Ref.52 Ref.53 Ref.54 Ref.55 Ref.59 Ref.60 Ref.61 Ref.65 Ref.66 Ref.67 Ref.68 Ref.72 Ref.75 Ref.78 Ref.79Jackson-Weiss syndrome (JWS) [MIM:123150]: An autosomal dominant craniosynostosis syndrome characterized by craniofacial abnormalities and abnormality of the feet: broad great toes with medial deviation and tarsal-metatarsal coalescence.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.53 Ref.55 Ref.59 Ref.64 Ref.67Apert syndrome (APRS) [MIM:101200]: A syndrome characterized by facio-cranio-synostosis, osseous and membranous syndactyly of the four extremities, and midface hypoplasia. The craniosynostosis is bicoronal and results in acrocephaly of brachysphenocephalic type. Syndactyly of the fingers and toes may be total (mitten hands and sock feet) or partial affecting the second, third, and fourth digits. Intellectual deficit is frequent and often severe, usually being associated with cerebral malformations.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.21 Ref.28 Ref.40 Ref.45 Ref.57 Ref.65 Ref.67 Ref.69 Ref.79Pfeiffer syndrome (PS) [MIM:101600]: A syndrome characterized by the association of craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly of the fingers and toes. Three subtypes are known: mild autosomal dominant form (type 1); cloverleaf skull, elbow ankylosis, early death, sporadic (type 2); craniosynostosis, early demise, sporadic (type 3).Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.31 Ref.40 Ref.48 Ref.56 Ref.58 Ref.59 Ref.63 Ref.65 Ref.70 Ref.71 Ref.73 Ref.74 Ref.75 Ref.79Beare-Stevenson cutis gyrata syndrome (BSTVS) [MIM:123790]: An autosomal dominant disease characterized by craniofacial anomalies, particularly craniosynostosis, and ear defects, cutis gyrata, acanthosis nigricans, anogenital anomalies, skin tags, and prominent umbilical stump. The skin furrows have a corrugated appearance and are widespread. Cutis gyrata variably affects the scalp, forehead, face, preauricular area, neck, trunk, hands, and feet.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.62 Ref.80Familial scaphocephaly syndrome (FSPC) [MIM:609579]: An autosomal dominant craniosynostosis syndrome characterized by scaphocephaly, macrocephaly, hypertelorism, maxillary retrusion, and mild intellectual disability. Scaphocephaly is the most common of the craniosynostosis conditions and is characterized by a long, narrow head. It is due to premature fusion of the sagittal suture or from external deformation.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.48 Ref.81Lacrimo-auriculo-dento-digital syndrome (LADDS) [MIM:149730]: An autosomal dominant ectodermal dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. Lacrimo-auriculo-dento-digital syndrome is characterized by aplastic/hypoplastic lacrimal and salivary glands and ducts, cup-shaped ears, hearing loss, hypodontia and enamel hypoplasia, and distal limb segments anomalies. In addition to these cardinal features, facial dysmorphism, malformations of the kidney and respiratory system and abnormal genitalia have been reported. Craniosynostosis and severe syndactyly are not observed.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.49 Ref.82Antley-Bixler syndrome, without genital anomalies or disordered steroidogenesis (ABS2) [MIM:207410]: A rare syndrome characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures. Arachnodactyly and/or camptodactyly have also been reported.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.77Bent bone dysplasia syndrome (BBDS) [MIM:614592]: A perinatal lethal skeletal dysplasia characterized by poor mineralization of the calvarium, craniosynostosis, dysmorphic facial features, prenatal teeth, hypoplastic pubis and clavicles, osteopenia, and bent long bones. Dysmorphic facial features included low-set ears, hypertelorism, midface hypoplasia, prematurely erupted fetal teeth, and micrognathia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.40 Ref.85

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.

Sequence caution: The sequence BAG57383.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

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