Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy.
Stem Cell Res
; 28: 56-60, 2018 04.
Article
in En
| MEDLINE
| ID: mdl-29427840
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cell Culture Techniques
/
Optic Atrophy, Hereditary, Leber
/
Induced Pluripotent Stem Cells
Limits:
Animals
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Female
/
Humans
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Middle aged
Language:
En
Journal:
Stem Cell Res
Year:
2018
Document type:
Article
Affiliation country:
Taiwan
Country of publication:
United kingdom