Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8425486 | Stem Cell Research | 2018 | 12 Pages |
Abstract
Leber's hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disease caused by homoplasmic point mutations in complex I subunit genes of mitochondrial DNA. In this report, we generated an induced pluripotent stem cell (iPSCs) line, TVGH-iPSC-010-09, from the peripheral blood mononuclear cells of a female patient with Leber's hereditary optic neuropathy (LHON) by using the Sendai-virus delivery system. The resulting iPSCs retained the disease-causing mitochondrial DNA mutation, expressed pluripotent markers and could differentiate into the three germ layers. We believe LHON patient-specific iPSCs provide a powerful in vitro model for evaluating the pathological phenotypes of the disease.
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Authors
Huai-En Lu, Yi-Ping Yang, Yan-Ting Chen, You-Ren Wu, Chia-Lin Wang, Fu-Ting Tsai, De-Kuang Hwang, Tai-Chi Lin, Shih-Jen Chen, An-Guor Wang, Patrick C.H. Hsieh, Shih-Hwa Chiou,