Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy.
Stem Cell Res
; 28: 56-60, 2018 04.
Article
em 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.
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Base de dados:
MEDLINE
Assunto principal:
Técnicas de Cultura de Células
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Atrofia Óptica Hereditária de Leber
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Células-Tronco Pluripotentes Induzidas
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article