TY - JOUR
T1 - Direct effects of mitochondrial dysfunction on poor bone health in Leigh syndrome
AU - Kato, Hiroki
AU - Han, Xu
AU - Yamaza, Haruyoshi
AU - Masuda, Keiji
AU - Hirofuji, Yuta
AU - Sato, Hiroshi
AU - Pham, Thanh Thi Mai
AU - Taguchi, Tomoaki
AU - Nonaka, Kazuaki
N1 - Funding Information:
We thank Dr. Toshio Kukita and all members of the Pediatric & Special Needs Dentistry at Kyushu University Hospital for valuable suggestions, technical support, and materials. We appreciate the technical assistance provided by the Research Support Center at the Research Center for Human Disease Modeling, Kyushu University Graduate School of Medical Sciences. This work was supported by the Japan Society for the Promotion of Science (KAKENHI grant numbers 25670877 and 16K15839 ), the Takeda Science Foundation and the Kaibara Morikazu Medical Science Promotion Foundation .
Publisher Copyright:
© 2017 Elsevier Inc.
PY - 2017/11/4
Y1 - 2017/11/4
N2 - Mitochondrial diseases are the result of aberrant mitochondrial function caused by mutations in either nuclear or mitochondrial DNA. Poor bone health has recently been suggested as a symptom of mitochondrial diseases; however, a direct link between decreased mitochondrial function and poor bone health in mitochondrial disease has not been demonstrated. In this study, stem cells from human exfoliated deciduous teeth (SHED) were isolated from a child with Leigh syndrome (LS), a mitochondrial disease, and the effects of decreased mitochondrial function on poor bone health were analyzed. Compared with control SHED, LS SHED displayed decreased osteoblastic differentiation and calcium mineralization. The intracellular and mitochondrial calcium levels were lower in LS SHED than in control SHED. Furthermore, the mitochondrial activity of LS SHED was decreased compared with control SHED both with and without osteoblastic differentiation. Our results indicate that decreased osteoblast differentiation potential and osteoblast function contribute to poor bone health in mitochondrial diseases.
AB - Mitochondrial diseases are the result of aberrant mitochondrial function caused by mutations in either nuclear or mitochondrial DNA. Poor bone health has recently been suggested as a symptom of mitochondrial diseases; however, a direct link between decreased mitochondrial function and poor bone health in mitochondrial disease has not been demonstrated. In this study, stem cells from human exfoliated deciduous teeth (SHED) were isolated from a child with Leigh syndrome (LS), a mitochondrial disease, and the effects of decreased mitochondrial function on poor bone health were analyzed. Compared with control SHED, LS SHED displayed decreased osteoblastic differentiation and calcium mineralization. The intracellular and mitochondrial calcium levels were lower in LS SHED than in control SHED. Furthermore, the mitochondrial activity of LS SHED was decreased compared with control SHED both with and without osteoblastic differentiation. Our results indicate that decreased osteoblast differentiation potential and osteoblast function contribute to poor bone health in mitochondrial diseases.
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U2 - 10.1016/j.bbrc.2017.09.045
DO - 10.1016/j.bbrc.2017.09.045
M3 - Article
C2 - 28899781
AN - SCOPUS:85029668902
SN - 0006-291X
VL - 493
SP - 207
EP - 212
JO - Biochemical and Biophysical Research Communications
JF - Biochemical and Biophysical Research Communications
IS - 1
ER -
