{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T03:43:19Z","timestamp":1790826199014,"version":"4.1.0"},"reference-count":55,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T00:00:00Z","timestamp":1757548800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["23-75-10006"],"award-info":[{"award-number":["23-75-10006"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is a severe X-linked recessive disorder caused by mutations in the DMD gene, leading to progressive muscle degeneration and fibrosis. A key pathological feature of DMD is mitochondrial dysfunction driven by calcium overload, which disrupts oxidative phosphorylation and triggers cell death pathways. This study shows the therapeutic potential of VBIT-4, a novel inhibitor of the mitochondrial voltage-dependent anion channel (VDAC), in two dystrophin-deficient mouse models: the mild mdx and the severe D2.DMDel8-34 strains. VBIT-4 administration (20 mg\/kg) reduced mitochondrial calcium overload, enhanced resistance to permeability transition pore induction, and improved mitochondrial ultrastructure in D2.DMDel8-34 mice, while showing negligible effects in mdx mice. VBIT-4 suppressed mitochondrial and total calpain activity and reduced endoplasmic reticulum stress markers, suggesting a role in mitigating proteotoxic stress. However, it did not restore oxidative phosphorylation or reduce oxidative stress. Functional assays revealed limited improvements in muscle strength and fibrosis reduction, exclusively in the severe model. These findings underscore VDAC as a promising target for severe DMD and highlight the critical role of mitochondrial calcium homeostasis in DMD progression.<\/jats:p>","DOI":"10.3390\/ijms26188845","type":"journal-article","created":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T10:50:04Z","timestamp":1757587804000},"page":"8845","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["VBIT-4 Rescues Mitochondrial Dysfunction and Reduces Skeletal Muscle Degeneration in a Severe Model of Duchenne Muscular Dystrophy"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7453-3390","authenticated-orcid":false,"given":"Mikhail V.","family":"Dubinin","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia E.","family":"Stepanova","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8054-0477","authenticated-orcid":false,"given":"Irina B.","family":"Mikheeva","sequence":"additional","affiliation":[{"name":"Laboratory of Experimental Neurobiology, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, 142290 Pushchino, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia D.","family":"Igoshkina","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-1495-634X","authenticated-orcid":false,"given":"Ekaterina N.","family":"Kraeva","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alena A.","family":"Cherepanova","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6227-7409","authenticated-orcid":false,"given":"Eugeny Yu.","family":"Talanov","sequence":"additional","affiliation":[{"name":"Laboratory of Mitochondrial Transport, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna V.","family":"Polikarpova","sequence":"additional","affiliation":[{"name":"Laboratory of Modeling and Gene Therapy of Hereditary Diseases, Institute of Gene Biology Russian Academy of Sciences, Vavilova 34\/5, 119334 Moscow, Russia"},{"name":"Marlin Biotech LLC, Triumfalny Ave. 1, Sirius, 354340 Sochi, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxim E.","family":"Astashev","sequence":"additional","affiliation":[{"name":"Federal Research Center \u201cPushchino Scientific Center for Biological Research of the Russian Academy of Sciences\u201d, Institute of Cell Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia"},{"name":"Prokhorov General Physics Institute, Russian Academy of Sciences, Vavilov Str. 38, 119991 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vyacheslav A.","family":"Loginov","sequence":"additional","affiliation":[{"name":"Laboratory of Modeling and Gene Therapy of Hereditary Diseases, Institute of Gene Biology Russian Academy of Sciences, Vavilova 34\/5, 119334 Moscow, Russia"},{"name":"Marlin Biotech LLC, Triumfalny Ave. 1, Sirius, 354340 Sochi, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3346-3242","authenticated-orcid":false,"given":"Tatiana V.","family":"Egorova","sequence":"additional","affiliation":[{"name":"Laboratory of Modeling and Gene Therapy of Hereditary Diseases, Institute of Gene Biology Russian Academy of Sciences, Vavilova 34\/5, 119334 Moscow, Russia"},{"name":"Marlin Biotech LLC, Triumfalny Ave. 1, Sirius, 354340 Sochi, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,9,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1038\/s41572-021-00248-3","article-title":"Duchenne muscular dystrophy","volume":"7","author":"Duan","year":"2021","journal-title":"Nat. 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