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Given the complexity of MD, in particular the primary mitochondrial respiratory chain (MRC) diseases due to oxidative phosphorylation (OXPHOS) dysfunction, a reliable single biomarker, relevant for the whole disease group, could be extremely difficult to find, most of times leading the physicians to better consider a \u2018biosignature\u2019 for the diagnosis, rather than a single biochemical marker. Serum biomarkers like lactate and pyruvate are largely determined in the diagnostic algorithm of MD, but they are not specific to this group of disorders. The concomitant determination of creatine (Cr), plasma amino acids, and urine organic acids might be helpful to reinforce the biosignature in some cases. In recent studies, serum fibroblast growth factor 21 (sFGF21) and serum growth differentiation factor 15 (sGDF15) appear to be promising molecules in identifying MD. Moreover, new different approaches have been developed to discover new MD biomarkers. This work discusses the most important biomarkers currently used in the diagnosis of MRC diseases, and some approaches under evaluation, discussing both their utility and weaknesses.<\/jats:p>","DOI":"10.1042\/ebc20170111","type":"journal-article","created":{"date-parts":[[2018,7,6]],"date-time":"2018-07-06T09:05:35Z","timestamp":1530867935000},"page":"443-454","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":68,"title":["Biomarkers for mitochondrial energy metabolism diseases"],"prefix":"10.1042","volume":"62","author":[{"given":"Sara","family":"Boenzi","sequence":"additional","affiliation":[{"name":"Division of Metabolism and Research Unit of Metabolic Biochemistry, Children\u2019s Hospital Bambino Ges\u00f9, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daria","family":"Diodato","sequence":"additional","affiliation":[{"name":"Division of Metabolism and Research Unit of Metabolic Biochemistry, Children\u2019s Hospital Bambino Ges\u00f9, Rome, Italy"},{"name":"Muscular and Neurodegenerative Disorders Unit and Laboratory of Molecular Medicine, Children\u2019s Hospital Bambino Ges\u00f9, Rome 49346, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2018,7,6]]},"reference":[{"key":"2021111610260676800_B1","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1067\/mcp.2001.113989","article-title":"Biomarkers and surrogate endpoints: preferred definitions and conceptual framework","volume":"69","author":"Biomarkers Definitions Working Group","year":"2001","journal-title":"Clin. 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