title: Patient-specific induced pluripotent stem cell models in mitochondrial diseases.
abstract: Mitochondrial diseases are clinical phenotypes associated with mitochondrial dysfunction, which can be caused by mutations of mitochondrial DNA (mtDNA) or of nuclear genes. Since there are no high-performance transfect systems yet to make particular mtDNA mutation, and tissue sources are limited by ethical issue and injury, the molecular pathogenesis of mitochondrial diseases remains poorly understood. The generation of induced pluripotent stem (iPS) cells from adult somatic cells has opened a remarkable avenue for theoretic study and therapeutic application. Patient-specific induced pluripotent stem cells and differentiated cells derived from them are attracting increasing attention to elucidate the mechanisms underlying mitochondrial diseases. In this review, we summarize the advances of iPS cells, advantages of patient- specific iPS cells as a novel disease model, especially in mitochondrial disease. Occurring challenges and perspectives of patient-specific iPS cells research are also discussed.
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhang
authors.forename: Shishi
authors.initials: S
authors.lastname: Li
authors.forename: Wei
authors.initials: W
authors.lastname: Yang
authors.forename: Dajiang
authors.initials: D
authors.lastname: Qin
authors.forename: Luyang
authors.initials: L
authors.lastname: Yu
authors.affiliation: Institute of Genetics, College of Life Sciences, Zhejiang University, 866 Yuhangtang Road, Hangzhou, Zhejiang 310058, P. R. China. qfyan@zju.edu.cn.
authors.forename: Qingfeng
authors.initials: Q
authors.lastname: Yan
mesh_terms: D000818:Animals; D002478:Cells, Cultured; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000473:pathology / Q000502:physiology*; D028361:Mitochondrial Diseases / Q000473:pathology*; D008954:Models, Biological