Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy

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

Stem Cell Research (Apr 2018)

Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy

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

Affiliations

Huai-En Lu: Bioresource Collection and Research Center, Food Industry Research and Development Institute, Hsinchu, Taiwan
Yi-Ping Yang: School of Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Neurosurgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
Yan-Ting Chen: Department of Ophthalmology, Changhua Christian Hospital, Changhua, Taiwan; Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan; Department of Optometry, Central Taiwan University of Science and Technology, Taichung, Taiwan
You-Ren Wu: Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan
Chia-Lin Wang: Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan
Fu-Ting Tsai: Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan
De-Kuang Hwang: Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan; School of Medicine, National Yang-Ming University, Taipei, Taiwan
Tai-Chi Lin: Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan; Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan
Shih-Jen Chen: Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan; School of Medicine, National Yang-Ming University, Taipei, Taiwan
An-Guor Wang: Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan; School of Medicine, National Yang-Ming University, Taipei, Taiwan
Patrick C.H. Hsieh: Institute of Biomedical Sciences, Academia Sinica, Taipei, Taiwan
Shih-Hwa Chiou: Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan; Department of Medical Research, Taipei Veterans General Hospital, Taipei, Taiwan; Genomic Research Center, Academia Sinica, Taipei, Taiwan; Corresponding author at: Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan.

Journal volume & issue: Vol. 28
pp. 56 – 60

Abstract

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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.

Published in Stem Cell Research

ISSN: 1873-5061 (Print); 1876-7753 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Science: Biology (General)
Website: https://www.journals.elsevier.com/stem-cell-research

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