2017
DOI: 10.18632/aging.101231 View full text |Buy / Rent full text
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Abstract: 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.

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“…This study demonstrated that iPSCs can be obtained from LHON- fibroblasts with normal efficiency, thus suggesting that iPSC reprogramming can tolerate a certain degree of OXPHOS defects. A successful iPSC model for translational medicine was obtained by Wong et al [ 31 ], who used cybrid technology to replace mutated mitochondrial DNA (mtDNA) from LHON-specific fibroblasts with wildtype mtDNA. The authors obtained mutation-free iPSCs and in these cybrid corrected iPSC-derived retinal ganglion cell, the cell death phenotype was res cued.…”
Section: Discussionmentioning
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“…This study demonstrated that iPSCs can be obtained from LHON- fibroblasts with normal efficiency, thus suggesting that iPSC reprogramming can tolerate a certain degree of OXPHOS defects. A successful iPSC model for translational medicine was obtained by Wong et al [ 31 ], who used cybrid technology to replace mutated mitochondrial DNA (mtDNA) from LHON-specific fibroblasts with wildtype mtDNA. The authors obtained mutation-free iPSCs and in these cybrid corrected iPSC-derived retinal ganglion cell, the cell death phenotype was res cued.…”
Section: Discussionmentioning
“…All these data reporting, selection, maturation and/or functional assessment of human PSC-derived RGCs should allow to study patient-derived iPSC lines, for a better understanding of ocular disease related to RGC impairment. In this context, different groups have already derived iPSCs from somatic cells of patients diagnosed for different optic neuropathies ( Chen et al, 2016 ; Ohlemacher et al, 2016 ; Teotia et al, 2017 ; Wong et al, 2017 ) ( Table 3 ). For example, the generation of LHON-patient iPSC allowed the exploration of oxidative phosphorylation defect ( Hung et al, 2016 ).…”
Section: Pluripotent-stem Cells-derived Rgcsmentioning
“…For example, the generation of LHON-patient iPSC allowed the exploration of oxidative phosphorylation defect ( Hung et al, 2016 ). In a more recent study, the same group demonstrated that replacing LHON mitochondrial DNA using cybrid technology in patient iPSCs can prevented the death of iPSC-derived RGCs ( Wong et al, 2017 ). Another iPSC line carrying mutation in the Optineurin ( OPTN ) gene has been recently derived ( Ohlemacher et al, 2016 ) and higher cell death of iPSC-derived RGCs carrying the mutation has been observed.…”
Section: Pluripotent-stem Cells-derived Rgcsmentioning
“…Induced pluripotent stem cells can now be routinely produced by reprogramming peripheral blood cells or fibroblasts obtained from a punch skin biopsy [ 43 ]. In parallel, differentiation protocols are being refined by a number of research groups worldwide to allow the efficient generation of various retinal cells, including RGCs, providing an ideal ‘disease in a dish’ model for testing promising drug molecules in a more physiological setting [ 44 46 ].…”
Section: Therapeutic Drug Screeningmentioning