FGFR3 blocking peptide

FGFR3 Antibody (C-term) Blocking Peptide

• Gene Names: FGFR3; ACH; CEK2; JTK4; CD333; HSFGFR3EX
• Synonyms: FGFR3; FGFR3 Antibody (C-term) Blocking Peptide; Fibroblast growth factor receptor 3; FGFR-3; CD333; JTK4; FGFR3 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the C-term region of human FGFR3. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
• Form/Format: The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.
• Sequence Length: 806

• Cellular Location: Isoform 1: Cell membrane; Single-pass type I membrane protein. Cytoplasmic vesicle. Endoplasmic reticulum Note: The activated receptor is rapidly internalized and degraded Detected in intracellular vesicles after internalization of the autophosphorylated receptor Isoform 3: Secreted.
• Tissue Location: Expressed in brain, kidney and testis. Very low or no expression in spleen, heart, and muscle. In 20- to 22- week old fetuses it is expressed at high level in kidney, lung, small intestine and brain, and to a lower degree in spleen, liver, and muscle. Isoform 2 is detected in epithelial cells. Isoform 1 is not detected in epithelial cells. Isoform 1 and isoform 2 are detected in fibroblastic cells.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for FGFR3 blocking peptide

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 and apoptosis. Plays an essential role in the regulation of chondrocyte differentiation, proliferation and apoptosis, and is required for normal skeleton development. Regulates both osteogenesis and postnatal bone mineralization by osteoblasts. Promotes apoptosis in chondrocytes, but can also promote cancer cell proliferation. Required for normal development of the inner ear. Phosphorylates PLCG1, CBL and FRS2. 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. Plays a role in the regulation of vitamin D metabolism. Mutations that lead to constitutive kinase activation or impair normal FGFR3 maturation, internalization and degradation lead to aberrant signaling. Over-expressed or constitutively activated FGFR3 promotes activation of PTPN11/SHP2, STAT1, STAT5A and STAT5B. Secreted isoform 3 retains its capacity to bind FGF1 and FGF2 and hence may interfere with FGF signaling.

NCBI and Uniprot Product Information

• NCBI GI #: 120050
• NCBI GeneID: 2261
• UniProt Accession #: P22607; Q14308; Q16294; Q16608; Q59FL9; D3DVP9; D3DVQ0
• Molecular Weight: 85,083 Da
• NCBI Official Full Name: Fibroblast growth factor receptor 3
• NCBI Official Synonym Full Names: fibroblast growth factor receptor 3
• NCBI Official Symbol: FGFR3
• NCBI Official Synonym Symbols: ACH; CEK2; JTK4; CD333; HSFGFR3EX
• NCBI Protein Information: fibroblast growth factor receptor 3
• UniProt Protein Name: Fibroblast growth factor receptor 3
• Protein Family: Fibroblast growth factor receptor
• UniProt Gene Name: FGFR3
• UniProt Synonym Gene Names: JTK4; FGFR-3
• UniProt Entry Name: FGFR3_HUMAN

NCBI Description

This gene encodes a member of the fibroblast growth factor receptor (FGFR) family, with its amino acid sequence being highly conserved between members and among divergent species. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein would consist 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 acidic and basic fibroblast growth hormone and plays a role in bone development and maintenance. Mutations in this gene lead to craniosynostosis and multiple types of skeletal dysplasia. Three alternatively spliced transcript variants that encode different protein isoforms have been described. [provided by RefSeq, Jul 2009]

Uniprot Description

FGFR3: a receptor tyrosine kinase of the highly-conserved FGFR family that binds fibroblast growth factor (FGF). Mutations are associated with thanatophoric dysplasia (TD), craniosynostosis Adelaide type, many craniosynostotic syndromes and bone malformations. Three splice-variant isoforms have been described. Activating point mutations cause dwarfism, including achondroplasia, hypochrondroplasia and thanatophoric dysplasia, and facial and other morphogenetic disorders, including Crouzon syndrome, craniosynostosis Adelaide type, San Diego skeletal displasia and Muenke syndrome. Translocations t(4;14) involving the IgH region are common in multiple myeloma and frequently involve FGFR3. Activated FGFR3 found in 30% of bladder cancers and several cervical cancers, but not in other tumors. Two mutations found in colorectal cancer.

Protein type: Membrane protein, integral; Protein kinase, TK; Kinase, protein; Protein kinase, tyrosine (receptor); EC 2.7.10.1; TK group; FGFR family

Chromosomal Location of Human Ortholog: 4p16.3

Cellular Component: endoplasmic reticulum; focal adhesion; Golgi apparatus; integral to plasma membrane; plasma membrane; transport vesicle

Molecular Function: 1-phosphatidylinositol-3-kinase activity; fibroblast growth factor binding; fibroblast growth factor receptor activity; phosphatidylinositol-4,5-bisphosphate 3-kinase activity; protein binding; protein-tyrosine kinase activity; Ras guanyl-nucleotide exchange factor activity

Biological Process: bone mineralization; chondrocyte differentiation; endochondral ossification; fibroblast growth factor receptor signaling pathway; JAK-STAT cascade; MAPKKK cascade; negative regulation of developmental growth; peptidyl-tyrosine phosphorylation; phosphoinositide-mediated signaling; positive regulation of cell proliferation; positive regulation of MAPKKK cascade; positive regulation of phosphoinositide 3-kinase activity; positive regulation of tyrosine phosphorylation of Stat1 protein; positive regulation of tyrosine phosphorylation of Stat3 protein; protein amino acid autophosphorylation; regulation of phosphoinositide 3-kinase cascade; skeletal development

Disease: Achondroplasia; Achondroplasia, Severe, With Developmental Delay And Acanthosis Nigricans; Bladder Cancer; Camptodactyly, Tall Stature, And Hearing Loss Syndrome; Cervical Cancer; Colorectal Cancer; Crouzon Syndrome With Acanthosis Nigricans; Hypochondroplasia; Lacrimoauriculodentodigital Syndrome; Muenke Syndrome; Nevus, Epidermal; Testicular Germ Cell Tumor; Thanatophoric Dysplasia, Type I; Thanatophoric Dysplasia, Type Ii