FGF23; ADHR; HYPF; HPDR2; PHPTC ; fgf 23

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

Monoclonal

IgG2

30F19

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-FGF-23 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.

The FGF family plays a central role during prenatal development and postnatal growth and regeneration of a variety of tissues, by promoting cellular proliferation and differentiation. FGF-23, FGF-21 and FGF-19 constitute an atypical FGF subfamily whose ligands act as circulating hormones and require the participation of a Klotho protein as a co-receptor for their signaling. FGF-23 is a bone-derived hormone that acts in the kidney to regulate phosphate homeostasis and vitamin D metabolism. The signaling receptor for FGF-23, a Klotho-FGFR1 (IIIc) complex, is an essential regulator of the renal sodium phosphate co-transporter and key vitamin D-metabolizing enzymes CYP27B1 and CYP24A1. Recombinant human FGF-23 is a 22.5 kDa globular protein containing 228 amino acid residues.

Western Blot (WB)

27,954 Da

fibroblast growth factor 23

fibroblast growth factor 23

Fibroblast growth factor 23

HYPF; FGF-23??[Similar Products]

This gene encodes a member of the fibroblast growth factor family of proteins, which possess broad mitogenic and cell survival activities and are involved in a variety of biological processes. The product of this gene regulates phosphate homeostasis and transport in the kidney. The full-length, functional protein may be deactivated via cleavage into N-terminal and C-terminal chains. Mutation of this cleavage site causes autosomal dominant hypophosphatemic rickets (ADHR). Mutations in this gene are also associated with hyperphosphatemic familial tumoral calcinosis (HFTC). [provided by RefSeq, Feb 2013]

FGF23: Regulator of phosphate homeostasis. Inhibits renal tubular phosphate transport by reducing SLC34A1 levels. Upregulates EGR1 expression in the presence of KL. Acts directly on the parathyroid to decrease PTH secretion. Regulator of vitamin-D metabolism. Negatively regulates osteoblast differentiation and matrix mineralization. Defects in FGF23 are the cause of autosomal dominant hypophosphataemic rickets (ADHR). ADHR is characterized by low serum phosphorus concentrations, rickets, osteomalacia, leg deformities, short stature, bone pain and dental abscesses. Defects in FGF23 are a cause of hyperphosphatemic familial tumoral calcinosis (HFTC). HFTC is a severe autosomal recessive metabolic disorder that manifests with hyperphosphatemia and massive calcium deposits in the skin and subcutaneous tissues. Belongs to the heparin-binding growth factors family.

Protein type: Cytokine; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 12p13.32

Cellular Component: extracellular region; extracellular space; Golgi lumen

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

Biological Process: fibroblast growth factor receptor signaling pathway; MAPKKK cascade; negative regulation of bone mineralization; negative regulation of hormone secretion; negative regulation of osteoblast differentiation; phosphate ion homeostasis; phosphoinositide-mediated signaling; regulation of phosphoinositide 3-kinase cascade; vitamin D catabolic process

Disease: Hypophosphatemic Rickets, Autosomal Dominant

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