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.
journal_name
Stem Cell Resjournal_title
Stem cell researchauthors
Lu HE,Yang YP,Chen YT,Wu YR,Wang CL,Tsai FT,Hwang DK,Lin TC,Chen SJ,Wang AG,Hsieh PCH,Chiou SHdoi
10.1016/j.scr.2018.01.029subject
Has Abstractpub_date
2018-04-01 00:00:00pages
56-60eissn
1873-5061issn
1876-7753pii
S1873-5061(18)30032-1journal_volume
28pub_type
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