The fibroblast growth factor receptors are members of the tyrosine kinase superfamily and consist of an extracellular ligand domain composed of three immunoglobulin-like domains, a single transmembrane helix domain, and an intracellular domain with tyrosine kinase activity. (Ornitz, DM. and Itoh, N) Monoclonal antibodies against the high affinity tyrosine kinase Fibroblast Growth Factor (FGF) receptor may help define receptor epitopes involved in FGF binding and signal transduction which mediate coronary and tumor angiogenesis. (Venkateswaran, S., et al).
Product Information
Format
Purified
Control
NIH/3T3 cell lysate
Presentation
Purified mouse monoclonal IgM in 0.015 M potassium phosphate buffer containing 0.15 M NaCl, 0.1% sodium azide, pH 7.20.
Applications
Application
Detect Fibroblast Growth Factor Receptor 1 using this Anti-Fibroblast Growth Factor Receptor 1 Antibody, clone VBS1 validated for use in WB, IP, IH(P), ELISA.
Key Applications
Western Blotting
ELISA
Immunohistochemistry (Paraffin)
Immunoprecipitation
Application Notes
Immunohistochemistry Analysis: A previous lot was used by an independent laboratory in IH(P) (Venkateswaran, S., et al)
Biological Information
Immunogen
FGF Receptor purified from bovine coronary venular endothelial cells.
Epitope
Extracellular
Clone
VBS1
Concentration
Please refer to the Certificate of Analysis for the lot-specific concentration.
Host
Mouse
Specificity
This antibody recognizes the extracellular ligand-binding domain of Fibroblast Growth Factor Receptor 1.
Isotype
IgM
Species Reactivity
Human Bovine Chicken Rat Mouse
Species Reactivity Note
Demonstrated to react with human, mouse, rat, cow and chicken.
FUNCTION:Receptor for basic fibroblast growth factor. Receptor for FGF23 in the presence of KL By similarity. A shorter form of the receptor could be a receptor for FGF1 (aFGF). CATALYTIC ACTIVITY:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. SUBUNIT STRUCTURE:Interacts with SHB. Interacts with KLB By similarity. Interacts with KL and FGF23 By similarity. 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. PTM:Binding of FGF1 and heparin promotes autophosphorylation on tyrosine residues and activation of the receptor. INVOLVEMENTI 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.
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.
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 non-syndromic trigonocephaly [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.
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.
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.
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.
Product Usage Statements
Quality Assurance
Evaluated by Western Blotting in NIH/3T3 cell lysate.
Western Blotting Analysis: 0.78 µg/mL of this antibody detected Fibroblast Growth Factor Receptor 1 in 10 µg of NIH/3T3 cell lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
The fibroblast growth factor receptors are members of the tyrosine kinase superfamily and consist of an extracellular ligand domain composed of three immunoglobulin-like domains, a single transmembrane helix domain, and an intracellular domain with tyrosine kinase activity. (Ornitz, DM. and Itoh, N) Monoclonal antibodies against the high affinity tyrosine kinase Fibroblast Growth Factor (FGF) receptor may help define receptor epitopes involved in FGF binding and signal transduction which mediate coronary and tumor angiogenesis. (Venkateswaran, S., et al).
Product Information
Format
Purified
Control
NIH/3T3 cell lysate
Presentation
Purified mouse monoclonal IgM in 0.015 M potassium phosphate buffer containing 0.15 M NaCl, 0.1% sodium azide, pH 7.20.
Applications
Application
Detect Fibroblast Growth Factor Receptor 1 using this Anti-Fibroblast Growth Factor Receptor 1 Antibody, clone VBS1 validated for use in WB, IP, IH(P), ELISA.
Key Applications
Western Blotting
ELISA
Immunohistochemistry (Paraffin)
Immunoprecipitation
Application Notes
Immunohistochemistry Analysis: A previous lot was used by an independent laboratory in IH(P) (Venkateswaran, S., et al)
Biological Information
Immunogen
FGF Receptor purified from bovine coronary venular endothelial cells.
Epitope
Extracellular
Clone
VBS1
Concentration
Please refer to the Certificate of Analysis for the lot-specific concentration.
Host
Mouse
Specificity
This antibody recognizes the extracellular ligand-binding domain of Fibroblast Growth Factor Receptor 1.
Isotype
IgM
Species Reactivity
Human Bovine Chicken Rat Mouse
Species Reactivity Note
Demonstrated to react with human, mouse, rat, cow and chicken.
FUNCTION:Receptor for basic fibroblast growth factor. Receptor for FGF23 in the presence of KL By similarity. A shorter form of the receptor could be a receptor for FGF1 (aFGF). CATALYTIC ACTIVITY:ATP + a [protein]-L-tyrosine = ADP + a [protein]-L-tyrosine phosphate. SUBUNIT STRUCTURE:Interacts with SHB. Interacts with KLB By similarity. Interacts with KL and FGF23 By similarity. 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. PTM:Binding of FGF1 and heparin promotes autophosphorylation on tyrosine residues and activation of the receptor. INVOLVEMENTI 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.
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.
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 non-syndromic trigonocephaly [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.
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.
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.
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.
Product Usage Statements
Quality Assurance
Evaluated by Western Blotting in NIH/3T3 cell lysate.
Western Blotting Analysis: 0.78 µg/mL of this antibody detected Fibroblast Growth Factor Receptor 1 in 10 µg of NIH/3T3 cell lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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