Eiko Nakamaru-Ogiso

Mitochondrial Medicine Frontier Program, The Children’s Hospital of Philadelphia

Affiliated withThe Children’s Hospital of Philadelphia University of Pennsylvania Perelman School of Medicine The Children's Hospital of Philadelphia

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Biography

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JoVE Journal Publications

ArticleTotal : 2	Year
Publication title Cited by 5	2021
Publication title	2026

Other Publications

Article	Year
Bacteria, yeast, worms, and flies: exploiting simple model organisms to investigate human mitochondrial diseases. Developmental disabilities research reviews\| PubMed ID: 20818735	2010
Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice. EMBO molecular medicine\| PubMed ID: 21567994	2011
NMNAT1 mutations cause Leber congenital amaurosis. Nature genetics\| PubMed ID: 22842227	2012
Primary respiratory chain disease causes tissue-specific dysregulation of the global transcriptome and nutrient-sensing signaling network. PloS one\| PubMed ID: 23894440	2013
Mitochondrial DNA variant in COX1 subunit significantly alters energy metabolism of geographically divergent wild isolates in Caenorhabditis elegans. Journal of molecular biology\| PubMed ID: 24534730	2014
Pharmacologic targeting of sirtuin and PPAR signaling improves longevity and mitochondrial physiology in respiratory chain complex I mutant Caenorhabditis elegans. Mitochondrion\| PubMed ID: 25744875	2015
Pharmacologic modeling of primary mitochondrial respiratory chain dysfunction in zebrafish. Neurochemistry international\| PubMed ID: 28732770	2018
Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease. Human molecular genetics\| PubMed ID: 30668749	2019
Regulation of nuclear epigenome by mitochondrial DNA heteroplasmy. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 31253706	2019
Combinatorial glucose, nicotinic acid, and N-acetylcysteine therapy has synergistic effect in preclinical C. elegans and zebrafish models of mitochondrial complex I disease. Human molecular genetics\| PubMed ID: 33640978	2021
The FusX TALE Base Editor (FusXTBE) for Rapid Mitochondrial DNA Programming of Human Cells and Zebrafish Disease Models . The CRISPR journal\| PubMed ID: 34847747	2021
Reduced ER-mitochondria connectivity promotes neuroblastoma multidrug resistance. The EMBO journal\| PubMed ID: 35211994	2022
Dichloroacetate improves mitochondrial function, physiology, and morphology in FBXL4 disease models. JCI insight\| PubMed ID: 35881484	2022
N-acetylcysteine and cysteamine bitartrate prevent azide-induced neuromuscular decompensation by restoring glutathione balance in two novel surf1-/- zebrafish deletion models of Leigh syndro... Human molecular genetics\| PubMed ID: 36795052	2023
-Glycosylation of MRS2 balances aerobic and anaerobic energy production by reducing rapid mitochondrial Mg influx in conditions of high glucose or impaired respiratory chain function. bioRxiv : the preprint server for biology\| PubMed ID: 39026824	2024
dldhcri3 zebrafish exhibit altered mitochondrial ultrastructure, morphology, and dysfunction partially rescued by probucol or thiamine. JCI insight\| PubMed ID: 39163131	2024
Zagociguat prevented stressor-induced neuromuscular dysfunction, improved mitochondrial physiology, and increased exercise capacity in diverse mitochondrial respiratory chain disease zebrafi... Frontiers in pharmacology\| PubMed ID: 40786033	2025
ndufs2 zebrafish have impaired survival, neuromuscular activity, morphology, and one-carbon metabolism treatable with folic acid. bioRxiv : the preprint server for biology\| PubMed ID: 40791373	2025
Roles of Subunit ND2/NuoN in the Proton Pumping Coupling Mechanism of Complex I. International journal of molecular sciences\| PubMed ID: 41977177	2026
Targeting the Mitochondrial Phenotype in Cockayne Syndrome Patient Cells: From Bioenergetic Fragility to Pharmacologic Rescue. bioRxiv : the preprint server for biology\| PubMed ID: 42232504	2026