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Author Kato, Hiroki ♦ Han, Xu ♦ Yamaza, Haruyoshi ♦ Masuda, Keiji ♦ Hirofuji, Yuta ♦ Sato, Hiroshi ♦ Pham, Thanh Thi Mai ♦ Taguchi, Tomoaki ♦ Nonaka, Kazuaki
Source United States Department of Energy Office of Scientific and Technical Information
Content type Text
Language English
Subject Keyword APPLIED LIFE SCIENCES ♦ CALCIUM ♦ CONNECTIVE TISSUE CELLS ♦ MINERALIZATION ♦ MITOCHONDRIA ♦ MUTATIONS ♦ SKELETON ♦ STEM CELLS ♦ SYMPTOMS ♦ TEETH
Abstract 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. - Highlights: • Stem cells from human exfoliated deciduous teeth (SHED) were isolated. • Osteoblast differentiation was decreased in Leigh syndrome (LS)-derived SHED. • Both intracellular and mitochondrial calcium levels were decreased in LS SHED. • Mitochondrial activity was also decreased in LS SHED. • Decreased mitochondrial activity directly contributes to poor bone health in LS.
ISSN 0006291X
Educational Use Research
Learning Resource Type Article
Publisher Date 2017-11-04
Publisher Place United States
Journal Biochemical and Biophysical Research Communications
Volume Number 493
Issue Number 1


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