Premium
Stem cells in reparing optic nerve damage
Author(s) -
Pebay A.,
Gill K.,
Needham K.,
van Bergen N.,
Lim S.,
Hernandez D.,
Liang H.,
Kearns L.,
Hung S.,
Hewitt A.,
Mackey D.,
Trounce I.,
Wong R.
Publication year - 2016
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2016.0026
Subject(s) - induced pluripotent stem cell , biology , human induced pluripotent stem cells , neuroscience , optic nerve , microbiology and biotechnology , phenotype , stem cell , mitochondrial dna , medicine , genetics , embryonic stem cell , gene
Summary Modelling Leber's Hereditary Optic neuropathy using human induced pluripotent stem cells. Human induced pluripotent stem cells (iPSCs) provide an invaluable tool for disease modelling in vitro . Here, we report the generation of iPSCs from LHON patients, their differentiation to retinal ganglion cells (RGCs) for subsequent modelling of phenotypic abnormalities. We also report the manipulation of mitochondrial DNA to correct LHON iPSCs by generation of cybrids. Comparison of LHON iPSCs and their isogenic cybrid control provide novel information on the potential cellular mechanisms leading to RGC death observed in human patients.