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Abnormal bone remodelling activity of dental follicle cells from a cleidocranial dysplasia patient
Author(s) -
Liu Yang,
Zhang Xianli,
Sun Xiangyu,
Wang Xiaozhe,
Zhang Chenying,
Zheng Shuguo
Publication year - 2018
Publication title -
oral diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.953
H-Index - 87
eISSN - 1601-0825
pISSN - 1354-523X
DOI - 10.1111/odi.12900
Subject(s) - rankl , dental follicle , chemistry , alkaline phosphatase , microbiology and biotechnology , missense mutation , osteoclast , western blot , osteoblast , acid phosphatase , dlx5 , cancer research , mutation , andrology , gene expression , pathology , biology , gene , medicine , biochemistry , receptor , mesenchymal stem cell , activator (genetics) , in vitro , enzyme , homeobox
Objectives To explore the role of dental follicle cells ( DFC s) with a novel cleidocranial dysplasia ( CCD ) causative gene RUNX 2 mutation ( DFC s RUNX2+/m ) in delayed permanent tooth eruption. Materials and methods A CCD patient with typical clinical features was involved in this study. DFC s RUNX2+/m were cultured and DNA was extracted for RUNX 2 mutation screening. Measurements of cell proliferation, alkaline phosphatase ( ALP ) activity, alizarin red staining and osteoblast‐specific genes expression were performed to assess osteogenesis of DFC s RUNX2+/m . Co‐culture of DFC s and peripheral blood mononuclear cells ( PBMC s), followed tartrate‐resistant acid phosphatase ( TRAP ) staining, real‐time PCR and western blot were performed to evaluate osteoclast‐inductive capacity of DFC s RUNX2+/m . Results A missense RUNX 2 mutation (c. 557G>C) was found in DFC s RUNX2+/m from the CCD patient. Compared with normal controls, this mutation did not affect the proliferation of DFC s RUNX2+/m , but down‐regulated the expression of osteogenesis‐related genes, leading to a decrease in ALP activity and mineralisation. Co‐culture results showed that DFC s RUNX2+/m reduced the formation of TRAP + multinucleated cells and the expression of osteoclastogenesis‐associated genes. Furthermore, the mutation reduced the ratio of RANKL / OPG in DFC s RUNX2+/m . Conclusions DFC s RUNX2+/m disturbs bone remodelling activity during tooth eruption through RANK / RANKL / OPG signalling pathway and may thus be responsible for impaired permanent tooth eruption in CCD patients.

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