Rabbit Anti-FGFR3 Polyclonal Antibody #abs130097

abs130097-50ug

1-2 Weeks

201

abs130097-100ug

1-2 Weeks

301

Description

FGFR3, a member of the FGFR family, is a receptor tyrosine kinase that binds fibroblast growth factor (FGF). It plays a crucial role in various developmental processes. Mutations in FGFR3 have been linked to several disorders, including thanatophoric dysplasia (TD), craniosynostosis Adelaide type, and various craniosynostotic syndromes and bone malformations.
FGFR3 has three splice-variant isoforms, each with its distinct functions and characteristics. When FGFR3 undergoes activating point mutations, it can lead to dwarfism-related conditions such as achondroplasia, hypochondroplasia, and thanatophoric dysplasia. Additionally, these mutations can cause facial and other morphogenetic disorders. Some examples of these disorders include Crouzon syndrome, craniosynostosis Adelaide type, San Diego skeletal dysplasia, and Muenke syndrome.
In certain cases of multiple myeloma, translocations involving the IgH region frequently involve FGFR3. This genetic rearrangement is commonly observed and plays a role in the development and progression of the disease.
Overall, FGFR3 is a critical receptor tyrosine kinase that is involved in various developmental processes and is associated with multiple disorders and conditions when mutated or involved in translocations.

Other names

The CD333 antigen, also known as ACH and CEK2, is a fibroblast growth factor receptor 3 (FGFR3) that is associated with achondroplasia and thanatophoric dwarfism. It is a heparin binding growth factor receptor that plays a role in the regulation of cell growth and differentiation. Other aliases for this protein include JTK4, SAM3, and MFR3. The FGFR3 protein is a tyrosine kinase that is involved in cell signaling pathways, and mutations in this gene can lead to various bone disorders. Additionally, FGFR3 has been identified as a potential therapeutic target for certain types of cancer.

Source

Rabbit

Specificity

The detection of endogenous levels of total FGFR3 can be achieved using an FGFR3 antibody.

Species Reactivity

Human;Mouse;Rat

Application

For Western Blotting, the suggested dilution ratio is 1:500 to 1:3000; for Immunohistochemistry, it is recommended to use a dilution ratio of 1:50 to 1:200; for Immunofluorescence/Immunocytochemistry, the dilution ratio of 1:100 to 1:500 is suggested; and for ELISA (peptide), a dilution ratio of 1:20000 to 1:40000 is recommended. Please make sure to follow these recommended dilutions for optimal results.

Immunogen

A synthesized peptide derived from human FGFR3.

Properties

Concentration

1mg/ml

Purification

Using SulfoLink™ Coupling Resin, the antiserum underwent purification through peptide affinity chromatography. A highly pure and concentrated preparation was obtained as a result of the rigorous purification process.

Clonality

Polyclonal Antibody

Stability & Storage

To maintain the integrity and efficacy of the material, it is vital to store it in optimal conditions. Therefore, it is recommended to keep it at a temperature of -20 °C for up to one year. To prevent any damage or deterioration, it is essential to avoid exposing the substance to repeated cycles of freezing and thawing.

Storage buffer

The Rabbit IgG is stored in a solution of phosphate buffered saline with a pH of 7.4, 150mM NaCl, 0.02% sodium azide, and 50% glycerol. To maintain its stability, it should be stored at a temperature of -20 °C and has a shelf life of 12 months from the time of receipt.

Target

Background

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.

Tissue specificity

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.

Posttranslational modification

Autophosphorylated. Binding of FGF family members together with heparan sulfate proteoglycan or heparin promotes receptor dimerization and autophosphorylation on tyrosine residues. Autophosphorylation occurs in trans between the two FGFR molecules present in the dimer. Phosphorylation at Tyr-724 is essential for stimulation of cell proliferation and activation of PIK3R1, STAT1 and MAP kinase signaling. Phosphorylation at Tyr-760 is required for interaction with PIK3R1 and PLCG1.Ubiquitinated. Is rapidly ubiquitinated after ligand binding and autophosphorylation, leading to receptor internalization and degradation. Subject to both proteasomal and lysosomal degradation.N-glycosylated in the endoplasmic reticulum. The N-glycan chains undergo further maturation to an Endo H-resistant form in the Golgi apparatus.

Celluar localization

Endoplasmic reticulum;Extracellular region or secreted;Golgi apparatus;Nucleus;Plasma Membrane;

UniPort

P22607