doi:10.1186/s13023-019-1128-zengEmperador, SoniaLópez-Gallardo, EsterHernández-Ainsa, CarmenHabbane, MounaMontoya, JulioBayona-Bafaluy, M. PilarRuiz-Pesini, EduardoKetogenic treatment reduces the percentage of a LHON heteroplasmic mutation and increases mtDNA amount of a LHON homoplasmic mutation.ART-2019-112595Background: The vision loss in Leber hereditary optic neuropathy patients is due to mitochondrial DNA mutations. No treatment has shown a clear-cut benefit on a clinically meaningful end-point. However, clinical evidences suggest two therapeutic approaches: the reduction of the mutation load in heteroplasmic patients or the elevation of mitochondrial DNA amount in homoplasmic patients. Results: Here we show that ketogenic treatment, in cybrid cell lines, reduces the percentage of the m.13094 T > C heteroplasmic mutation and also increases the mitochondrial DNA levels of the m.11778G > A mitochondrial genotype. Conclusions: These results suggest that ketogenic diet could be a therapeutic strategy for Leber hereditary optic neuropathy.2019http://zaguan.unizar.es/record/8988410.1186/s13023-019-1128-zhttp://zaguan.unizar.es/record/89884oai:zaguan.unizar.es:89884info:eu-repo/grantAgreement/ES/DGA/B33-17Rinfo:eu-repo/grantAgreement/ES/ISCIII/PI17-00021info:eu-repo/grantAgreement/ES/ISCIII/PI17-00166Orphanet Journal of Rare Diseases 14 (2019), 150 [6 pp.]byhttp://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccess