Cell therapy using retinal progenitor cells shows therapeutic effect in a chemically-induced rotenone mouse model of Leber hereditary optic neuropathy

Primary mitochondrial disorders occur at a prevalence of one in 10 000; ∼50% of these demonstrate ocular pathology. Leber hereditary optic neuropathy (LHON) is the most common primary mitochondrial disorder. LHON results from retinal ganglion cell pathology, which leads to optic nerve degeneration a...

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Veröffentlicht in:	European journal of human genetics : EJHG 2014-11, Vol.22 (11), p.1314-1320
Hauptverfasser:	Mansergh, Fiona C, Chadderton, Naomi, Kenna, Paul F, Gobbo, Oliviero L, Farrar, G Jane
Format:	Artikel
Sprache:	eng
Schlagworte:	Adeno-associated virus Animal models Animals Apoptosis Blindness Cell death Cell- and Tissue-Based Therapy - methods Cells, Cultured Degeneration Dependovirus - genetics Disease Disease Models, Animal Electron transport chain Electron Transport Complex I - genetics Electron Transport Complex I - metabolism Gene therapy Genetic Markers Glaucoma Injection Magnetic Resonance Imaging Manganese Mice Mitochondria Mitochondria - genetics Mitochondrial DNA Mutation N.M.R Neural stem cells Neuropathy NMR Nuclear magnetic resonance Optic Atrophy, Hereditary, Leber - chemically induced Optic Atrophy, Hereditary, Leber - genetics Optic Atrophy, Hereditary, Leber - therapy Optic nerve Optic Nerve - drug effects Optic Nerve - pathology Optic neuropathy Pathology Photoreceptors Retina Retina - cytology Retinal Ganglion Cells - cytology Retinal Ganglion Cells - drug effects Retinal Ganglion Cells - pathology Rodents Rotenone Rotenone - toxicity Saccharomyces cerevisiae Proteins - genetics Saccharomyces cerevisiae Proteins - metabolism Stem Cell Transplantation Stem cells Stem Cells - cytology Visual perception
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