{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T05:42:52Z","timestamp":1788846172378,"version":"build-2803163510"},"reference-count":51,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T00:00:00Z","timestamp":1661299200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Office of Research Infrastructure Programs, National Institutes of Health","award":["P40 OD010440"],"award-info":[{"award-number":["P40 OD010440"]}]},{"name":"Office of Research Infrastructure Programs, National Institutes of Health","award":["JP22zf0127001"],"award-info":[{"award-number":["JP22zf0127001"]}]},{"name":"Office of Research Infrastructure Programs, National Institutes of Health","award":["AMED-CREST 16814305"],"award-info":[{"award-number":["AMED-CREST 16814305"]}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["P40 OD010440"],"award-info":[{"award-number":["P40 OD010440"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP22zf0127001"],"award-info":[{"award-number":["JP22zf0127001"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["AMED-CREST 16814305"],"award-info":[{"award-number":["AMED-CREST 16814305"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mitochonic Acid 5 (MA-5) enhances mitochondrial ATP production, restores fibroblasts from mitochondrial disease patients and extends the lifespan of the disease model \u201cMitomouse\u201d. Additionally, MA-5 interacts with mitofilin and modulates the mitochondrial inner membrane organizing system (MINOS) in mammalian cultured cells. Here, we used the nematode Caenorhabditis elegans to investigate whether MA-5 improves the Duchenne muscular dystrophy (DMD) model. Firstly, we confirmed the efficient penetration of MA-5 in the mitochondria of C. elegans. MA-5 also alleviated symptoms such as movement decline, muscular tone, mitochondrial fragmentation and Ca2+ accumulation of the DMD model. To assess the effect of MA-5 on mitochondria perturbation, we employed a low concentration of rotenone with or without MA-5. MA-5 significantly suppressed rotenone-induced mitochondria reactive oxygen species (ROS) increase, mitochondrial network fragmentation and nuclear destruction in body wall muscles as well as endogenous ATP levels decline. In addition, MA-5 suppressed rotenone-induced degeneration of dopaminergic cephalic (CEP) neurons seen in the Parkinson\u2019s disease (PD) model. Furthermore, the application of MA-5 reduced mitochondrial swelling due to the immt-1 null mutation. These results indicate that MA-5 has broad mitochondrial homing and MINOS stabilizing activity in metazoans and may be a therapeutic agent for these by ameliorating mitochondrial dysfunction in DMD and PD.<\/jats:p>","DOI":"10.3390\/ijms23179572","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T21:03:51Z","timestamp":1661375031000},"page":"9572","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Mitochonic Acid 5 Improves Duchenne Muscular Dystrophy and Parkinson\u2019s Disease Model of Caenorhabditis elegans"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7635-6471","authenticated-orcid":false,"given":"Xintong","family":"Wu","sequence":"first","affiliation":[{"name":"Graduate School of Life Sciences, Tohoku University, Sendai 980-8577, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoi","family":"Nagasawa","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences, Tohoku University, Sendai 980-8577, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kasumi","family":"Muto","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences, Tohoku University, Sendai 980-8577, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maiko","family":"Ueda","sequence":"additional","affiliation":[{"name":"Biomedical Research Core, Tohoku University Graduate School of Medicine, Sendai 980-0872, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chitose","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Clinical Biology and Hormonal Regulation, Tohoku University Graduate School of Medicine, Sendai 980-0872, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takaaki","family":"Abe","sequence":"additional","affiliation":[{"name":"Department of Clinical Biology and Hormonal Regulation, Tohoku University Graduate School of Medicine, Sendai 980-0872, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6920-0594","authenticated-orcid":false,"given":"Atsushi","family":"Higashitani","sequence":"additional","affiliation":[{"name":"Graduate School of Life Sciences, Tohoku University, Sendai 980-8577, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1620\/tjem.236.225","article-title":"Mitochonic Acid 5 (MA-5), a Derivative of the Plant Hormone Indole-3-Acetic Acid, Improves Survival of Fibroblasts from Patients with Mitochondrial Diseases","volume":"236","author":"Suzuki","year":"2015","journal-title":"Tohoku J. 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