Human Total FGFR3 ELISA Kit PicoKine®

Fibroblast growth factor receptor 3, also known as CD333, is a protein that in humans is encoded by the FGFR3 gene. 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. The FGFR3 gene is mapped to the HD region on chromosome 4p16.3. 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.

Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 465 297 377 495 409 10.05 2.5% Sample 2 1948 1228 1440 1005 1405 72.51 5.2% Sample 3 6157 7228 5165 7566 6529 348.65 5.3% Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 465 297 377 495 409 10.05 2.5% Sample 1 465 297 377 495 409 10.05 2.5% Sample 2 1948 1228 1440 1005 1405 72.51 5.2% Sample 2 1948 1228 1440 1005 1405 72.51 5.2% Sample 3 6157 7228 5165 7566 6529 348.65 5.3% Sample 3 6157 7228 5165 7566 6529 348.65 5.3%

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. .

Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 465 1948 6157 490 1119 6839 Standard deviation 18.74 39.74 363.26 29.74 65.13 457.53 CV (%) 4.0% 2.0% 5.9% 6.1% 5.8% 6.7% Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 465 1948 6157 490 1119 6839 Standard deviation 18.74 39.74 363.26 29.74 65.13 457.53 CV (%) 4.0% 2.0% 5.9% 6.1% 5.8% 6.7% Intra-Assay Precision Inter-Assay Precision Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 n 16 16 16 24 24 24 Mean (pg/ml) 465 1948 6157 490 1119 6839 Mean (pg/ml) 465 1948 6157 490 1119 6839 Standard deviation 18.74 39.74 363.26 29.74 65.13 457.53 Standard deviation 18.74 39.74 363.26 29.74 65.13 457.53 CV (%) 4.0% 2.0% 5.9% 6.1% 5.8% 6.7% CV (%) 4.0% 2.0% 5.9% 6.1% 5.8% 6.7%

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. .

Description|Quantity Pre-coated 96-well strip microplate|1 Standard|2 vials Biotinylated antibody (100x) |100ul Avidin-Biotin-Peroxidase Complex (100x) |100ul Sample Diluent|30ml Antibody Diluent|12ml Avidin-Biotin-Peroxidase Diluent|12ml Color Developing Reagent (TMB) |10ml Stop Solution|10ml Wash Buffer (25x) |20ml Adhesive plate sealers|4

Store at 4°C for 6 months, at -20°C for 12 months. Avoid multiple freeze-thaw cycles (Ships with gel ice, can store for up to 3 days in room temperature. Freeze upon receiving.)

Isoform 1: Cell membrane; Single-pass type I membrane protein. Cytoplasmic vesicle. Endoplasmic reticulum. The activated receptor is rapidly internalized and degraded. Detected in intracellular vesicles after internalization of the autophosphorylated receptor.

Concentration (pg/ml) 0 250 500 1000 2000 4000 8000 16000 O.D. 0.027 0.083 0.149 0.26 0.452 0.852 1.347 1.965 Concentration (pg/ml) 0 250 500 1000 2000 4000 8000 16000 O.D. 0.027 0.083 0.149 0.26 0.452 0.852 1.347 1.965 Concentration (pg/ml) 0 250 500 1000 2000 4000 8000 16000 Concentration (pg/ml) 0 250 500 1000 2000 4000 8000 16000 O.D. 0.027 0.083 0.149 0.26 0.452 0.852 1.347 1.965 O.D. 0.027 0.083 0.149 0.26 0.452 0.852 1.347 1.965