Abstract: Phosphate- regulating gene with homologies to endopeptidase, on the X- chromosome（PHEX） is tightly correlated with renal phosphate homeostasis and bone, dentin mineralization. PHEX mutations could cause morphological
abnormality severely of bone and dentin, such as rickets, interglobular dentin, widen predentin and so on. This review summarizes the possible mechanisms of PHEX involving biological mineralization and phosphate metabolic homeostasis.