{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T04:59:54Z","timestamp":1780981194018,"version":"3.54.1"},"reference-count":42,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T00:00:00Z","timestamp":1780617600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Primary mitochondrial diseases (PMD) are rare disorders with limited therapeutic options. Coenzyme Q10 (CoQ10) supplementation is widely used, although formulation differences can affect absorption and efficacy. This open-label pilot feasibility trial evaluated a food for special medical purposes (FSMP) containing high-dose CoQ10 (250 mg per capsule) in patients with PMD. Ten patients (mean age: 55.5 \u00b1 8.6 years) were enrolled. Serum\/plasma biomarkers, including CoQ10, fibroblast growth factor 21 (FGF21), growth differentiation factor 15 (GDF15), ferric-reducing antioxidant power (FRAP), total sulfhydryl groups (t-SH), and advanced oxidation protein products (AOPP), were assessed at baseline (T0, after \u226530 days of conventional ubidecarenone) and after 30 days of FSMP administration (T1). Fatigue severity scale (FSS) and 5-times sit-to-stand test (5xSST) were evaluated at both timepoints. FSMP was administered at 250 or 500 mg\/day. Twenty sex- and age-matched healthy controls were included for CoQ10 comparison. Absolute CoQ10 concentrations remained stable overall at T1, with all patients maintaining levels above 390 ng\/mL (100% vs. 60% at T0), although concentrations remained lower than in healthy controls (p &lt; 0.01). Dose-normalized CoQ10 exposure was significantly higher with FSMP versus conventional ubidecarenone (p &lt; 0.001, Cohen\u2019s d = 7.31). FGF21, GDF15, AOPP, and t-SH remained unchanged, whereas FRAP increased at T1 (p &lt; 0.01). No significant changes were observed in 5xSST and FSS. Exploratory analyses indicated inter-individual variability in functional responses. FSMP was associated with higher dose-normalized systemic CoQ10 exposure, more consistent circulating CoQ10, and increased FRAP levels. Its simplified dosing regimen may support long-term adherence. Larger studies are warranted to validate these preliminary findings.<\/jats:p>","DOI":"10.3390\/ijms27115127","type":"journal-article","created":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T17:18:16Z","timestamp":1780679896000},"page":"5127","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Evaluating a Coenzyme Q10-Based Food for Special Medical Purpose, for Mitochondrial Diseases Management: An Open-Label, Pilot Trial"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5832-7098","authenticated-orcid":false,"given":"Lucia","family":"Chico","sequence":"first","affiliation":[{"name":"Laboratori Aliveda S.r.l., Crespina Lorenzana, 56042 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piervito","family":"Lopriore","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"},{"name":"Ph.D. School in Translational Medicine, Scuola Superiore Sant\u2019Anna, 56127 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Cecchi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana","family":"Meli","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Bernardini","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Balestrini","sequence":"additional","affiliation":[{"name":"Laboratori Aliveda S.r.l., Crespina Lorenzana, 56042 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maico","family":"Polzella","sequence":"additional","affiliation":[{"name":"Laboratori Aliveda S.r.l., Crespina Lorenzana, 56042 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7548-7699","authenticated-orcid":false,"given":"Vincenzo","family":"Montano","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2738-8562","authenticated-orcid":false,"given":"Michelangelo","family":"Mancuso","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Neurological Institute, University of Pisa, 56100 Pisa, Italy"},{"name":"CTMM\u2014Interdepartmental Research Centre for Translational Medicine in Neuromuscular and Mitochondrial Diseases, University of Pisa, 56100 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1146\/annurev.pathol.4.110807.092314","article-title":"Mitochondrial Energetics and Therapeutics","volume":"5","author":"Wallace","year":"2010","journal-title":"Annu. 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