Compromised alveolar bone cells in a patient with dentinogenesis imperfecta caused by DSPP mutation

Abstract

DSPP mutation can induce the behavior alterations of alveolar bone cells.

Haploinsufficiency of Dspp Gene Causes Dentin Dysplasia Type II in Mice

“…Dentin sialophosphoprotein was initially found to be specifically expressed by odontoblasts and transiently by pre-ameloblasts ( D’Souza et al, 1992 ; Begue-Kirn et al, 1998 ), while it was later observed also in bone ( Qin et al, 2002 ), periodontium ( Baba et al, 2004 ) and other non-mineralized tissues ( Ogbureke and Fisher, 2004 , 2007 ; Sun et al, 2010 ; Prasad et al, 2011 ). Patients with DGI have compromised alveolar bone cells ( Porntaveetus et al, 2019 ), suggesting that these patients may suffer from periodontal diseases. DGI-III animal model ( Dspp null mice) developed severe periodontitis, also displayed impaired cranial bone development and long bone mineralization ( Verdelis et al, 2008 ; Gibson et al, 2013b ; Chen et al, 2015 ).…”

Vacuolar protein sorting 4B regulates the proliferation and odontoblastic differentiation of human dental pulp stem cells through the Wnt-β-catenin signalling pathway

“…To this end, we performed ALP and ARS staining to monitor ALP activity and mineralization, respectively, and we examined the levels of expression of odontoblastic differentiation markers DSPP, BSP, ALP and OPN. DSPP is the primary noncollagenous protein in tooth dentin and has long been regarded as a specific marker of dentinogenesis [24]. BSP is an acidic noncollagenous glycoprotein abundantly expressed in mineralized tissues and performs an essential function in the initial mineralization of bone, dentin and cementum [25].…”

Precision medicine in Thailand

“…Patients consulted for diagnosis by NGS are from all specialties, including neurologists (Veeravigrom et al, ), dentists (Intarak, Theerapanon, Ittiwut, et al, ; Nowwarote et al, ; Porntaveetus, Nowwarote, et al, b; Porntaveetus, Osathanon, et al, ), orthopedists, endocrinologists (Sangsin, Srichomthong, Pongpanich, Suphapeetiporn, & Shotelersuk, , ), hematologists (Ittiwut et al, ; Ittiwut et al, ), dermatologists (Intarak, Theerapanon, Srijunbarl, et al, ; Panmontha et al, ; Panmontha et al, ), immunologists (Suratannon et al, ), syndromologists (Porntaveetus, Abid, et al, ; Porntaveetus, Srichomthong, Ohazama, Suphapeetiporn, & Shotelersuk, ; Porntaveetus, Theerapanon, Srichomthong, & Shotelersuk, ), biochemical geneticists (Chaiyasap et al, ; Phowthongkum, Ittiwut, & Shotelersuk, ; Porntaveetus, Srichomthong, Suphapeetiporn, & Shotelersuk, ), oncologists (Sahakitrungruang et al, ), and radiologists (Yeetong, Phewplung, Kamolvisit, Suphapeetiporn, & Shotelersuk, ). We have observed a wide range of yields depending on disease groups, recruitment criteria and, very importantly the cooperation of the clinicians and the bioinformaticians.…”