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大学・研究所にある論文を検索できる 「リー症候群における骨障害に対するミトコンドリア機能障害の直接的な影響」の論文概要。リケラボ論文検索は、全国の大学リポジトリにある学位論文・教授論文を一括検索できる論文検索サービスです。
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リー症候群における骨障害に対するミトコンドリア機能障害の直接的な影響

韓, 旭 HAN, XU カン, キョク 九州大学

2020.09.25

概要

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 (Ctrl-SHED), LS-SHED displayed decreased osteoblastic differentiation and calcium mineralization. The intracellular and mitochondrial calcium levels were lower in LS-SHED than in Ctrl- SHED. Furthermore, the mitochondrial activity of LS-SHED was decreased compared with Ctrl-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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