Direct effects of mitochondrial dysfunction on poor bone health in Leigh syndrome

Hiroki Kato, Xu Han, Haruyoshi Yamaza, Keiji Masuda, Yuta Hirofuji, Hiroshi Sato, Thanh Thi Mai Pham, Tomoaki Taguchi, Kazuaki Nonaka

研究成果: ジャーナルへの寄稿記事

8 引用 (Scopus)

抄録

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.

元の言語英語
ページ(範囲)207-212
ページ数6
ジャーナルBiochemical and Biophysical Research Communications
493
発行部数1
DOI
出版物ステータス出版済み - 11 4 2017

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Leigh Disease
Mitochondrial Diseases
Bone
Health
Bone and Bones
Osteoblasts
Calcium
Deciduous Tooth
Stem cells
Mitochondrial DNA
Stem Cells
Mutation

All Science Journal Classification (ASJC) codes

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

これを引用

Direct effects of mitochondrial dysfunction on poor bone health in Leigh syndrome. / Kato, Hiroki; Han, Xu; Yamaza, Haruyoshi; Masuda, Keiji; Hirofuji, Yuta; Sato, Hiroshi; Pham, Thanh Thi Mai; Taguchi, Tomoaki; Nonaka, Kazuaki.

:: Biochemical and Biophysical Research Communications, 巻 493, 番号 1, 04.11.2017, p. 207-212.

研究成果: ジャーナルへの寄稿記事

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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

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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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