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Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy
Cybrid technology was used to replace Leber hereditary optic neuropathy (LHON) causing mitochondrial DNA (mtDNA) mutations from patient-specific fibroblasts with wildtype mtDNA, and mutation-free induced pluripotent stem cells (iPSCs) were generated subsequently. Retinal ganglion cell (RGC) differen...
| Autores principales: | , , , , , , , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
Impact Journals LLC
2017
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5425131/ https://www.ncbi.nlm.nih.gov/pubmed/28455970 http://dx.doi.org/10.18632/aging.101231 |
| _version_ | 1783235257639632896 |
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| author | Wong, Raymond C.B. Lim, Shiang Y. Hung, Sandy S.C. Jackson, Stacey Khan, Shahnaz Van Bergen, Nicole J. De Smit, Elisabeth Liang, Helena H. Kearns, Lisa S Clarke, Linda Mackey, David A. Hewitt, Alex W. Trounce, Ian A. Pébay, Alice |
| author_facet | Wong, Raymond C.B. Lim, Shiang Y. Hung, Sandy S.C. Jackson, Stacey Khan, Shahnaz Van Bergen, Nicole J. De Smit, Elisabeth Liang, Helena H. Kearns, Lisa S Clarke, Linda Mackey, David A. Hewitt, Alex W. Trounce, Ian A. Pébay, Alice |
| author_sort | Wong, Raymond C.B. |
| collection | PubMed |
| description | Cybrid technology was used to replace Leber hereditary optic neuropathy (LHON) causing mitochondrial DNA (mtDNA) mutations from patient-specific fibroblasts with wildtype mtDNA, and mutation-free induced pluripotent stem cells (iPSCs) were generated subsequently. Retinal ganglion cell (RGC) differentiation demonstrates increased cell death in LHON-RGCs and can be rescued in cybrid corrected RGCs. |
| format | Online Article Text |
| id | pubmed-5425131 |
| institution | National Center for Biotechnology Information |
| language | English |
| publishDate | 2017 |
| publisher | Impact Journals LLC |
| record_format | MEDLINE/PubMed |
| spelling | pubmed-54251312017-05-11 Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy Wong, Raymond C.B. Lim, Shiang Y. Hung, Sandy S.C. Jackson, Stacey Khan, Shahnaz Van Bergen, Nicole J. De Smit, Elisabeth Liang, Helena H. Kearns, Lisa S Clarke, Linda Mackey, David A. Hewitt, Alex W. Trounce, Ian A. Pébay, Alice Aging (Albany NY) Research Paper Cybrid technology was used to replace Leber hereditary optic neuropathy (LHON) causing mitochondrial DNA (mtDNA) mutations from patient-specific fibroblasts with wildtype mtDNA, and mutation-free induced pluripotent stem cells (iPSCs) were generated subsequently. Retinal ganglion cell (RGC) differentiation demonstrates increased cell death in LHON-RGCs and can be rescued in cybrid corrected RGCs. Impact Journals LLC 2017-04-29 /pmc/articles/PMC5425131/ /pubmed/28455970 http://dx.doi.org/10.18632/aging.101231 Text en Copyright: © 2017 Wong et al. http://creativecommons.org/licenses/by/3.0/ This article is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0/) (CC-BY), which permits unrestricted use and redistribution provided that the original author and source are credited. |
| spellingShingle | Research Paper Wong, Raymond C.B. Lim, Shiang Y. Hung, Sandy S.C. Jackson, Stacey Khan, Shahnaz Van Bergen, Nicole J. De Smit, Elisabeth Liang, Helena H. Kearns, Lisa S Clarke, Linda Mackey, David A. Hewitt, Alex W. Trounce, Ian A. Pébay, Alice Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title_full | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title_fullStr | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title_full_unstemmed | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title_short | Mitochondrial replacement in an iPSC model of Leber's hereditary optic neuropathy |
| title_sort | mitochondrial replacement in an ipsc model of leber's hereditary optic neuropathy |
| topic | Research Paper |
| url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5425131/ https://www.ncbi.nlm.nih.gov/pubmed/28455970 http://dx.doi.org/10.18632/aging.101231 |
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