SPR4-peptide alters bone metabolism of normal and HYP mice

摘要

Context: ASARM-peptides are substrates and ligands for PHEX, the gene responsible for X-linked hypophosphatemic rickets (HYP). PHEX binds to the DMP1-ASARM-motif to form a trimeric-complex with alpha(5)beta(3)-integrin on the osteocyte surface and this suppresses FGF23 expression. ASARM-peptide disruption of this complex increases FGF23 expression. We used a 4.2 kDa peptide (SPR4) that binds to ASARM-peptide and ASARM-motif to study DMPl-PHEX interactions and to assess SPR4 for treating inherited hypophosphatemic rickets.