{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T04:31:38Z","timestamp":1786422698734,"version":"build-2736575974"},"reference-count":40,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/501100007937","name":"Peking University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007937","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinica Chimica Acta"],"published-print":{"date-parts":[[2026,9]]},"DOI":"10.1016\/j.cca.2026.121172","type":"journal-article","created":{"date-parts":[[2026,6,14]],"date-time":"2026-06-14T18:01:25Z","timestamp":1781460085000},"page":"121172","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"special_numbering":"C","title":["Detection of NADH\/NAD+ dysregulation in MELAS via diazo-carboxyl click derivatization mass spectrometry"],"prefix":"10.1016","volume":"591","author":[{"given":"Jiaqi","family":"Hu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tongling","family":"Liufu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cong","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qijin","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meng","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yun","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Quan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhixing","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaoxia","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1\u20132","key":"10.1016\/j.cca.2026.121172_bb0005","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.ymgme.2015.06.004","article-title":"MELAS syndrome: clinical manifestations, pathogenesis, and treatment options","volume":"116","author":"El-Hattab","year":"2015","journal-title":"Mol. Genet. Metab."},{"issue":"1","key":"10.1016\/j.cca.2026.121172_bb0010","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1186\/s12887-020-1912-x","article-title":"Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I","volume":"20","author":"Danhelovska","year":"2020","journal-title":"BMC Pediatr."},{"issue":"2","key":"10.1016\/j.cca.2026.121172_bb0015","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/0960-8966(92)90045-8","article-title":"Melas: an original case and clinical criteria for diagnosis","volume":"2","author":"Hirano","year":"1992","journal-title":"Neuromuscul. Disord."},{"key":"10.1016\/j.cca.2026.121172_bb0020","doi-asserted-by":"crossref","DOI":"10.1016\/j.jns.2019.116499","article-title":"Patients with MELAS with negative myopathology for characteristic ragged-red fibers","volume":"408","author":"Lu","year":"2020","journal-title":"J. Neurol. Sci."},{"issue":"3 Pt 1","key":"10.1016\/j.cca.2026.121172_bb0025","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1212\/WNL.42.3.545","article-title":"Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation","volume":"42","author":"Goto","year":"1992","journal-title":"Neurology"},{"issue":"5 Pt A","key":"10.1016\/j.cca.2026.121172_bb0030","doi-asserted-by":"crossref","first-page":"1596","DOI":"10.1016\/j.bbadis.2018.02.005","article-title":"The accumulation of assembly intermediates of the mitochondrial complex I matrix arm is reduced by limiting glucose uptake in a neuronal-like model of MELAS syndrome","volume":"1864","author":"Geffroy","year":"2018","journal-title":"Biochim. Biophys. Acta Mol. basis Dis."},{"issue":"38","key":"10.1016\/j.cca.2026.121172_bb0035","doi-asserted-by":"crossref","first-page":"E3622","DOI":"10.1073\/pnas.1311660110","article-title":"Tissue- and cell-type-specific manifestations of heteroplasmic mtDNA 3243A>G mutation in human induced pluripotent stem cell-derived disease model","volume":"110","author":"H\u00e4m\u00e4l\u00e4inen","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"45","key":"10.1016\/j.cca.2026.121172_bb0040","doi-asserted-by":"crossref","first-page":"12715","DOI":"10.1073\/pnas.1609227113","article-title":"Spatial dynamics of SIRT1 and the subnuclear distribution of NADH species","volume":"113","author":"Aguilar-Arnal","year":"2016","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.cca.2026.121172_bb0045","doi-asserted-by":"crossref","first-page":"552","DOI":"10.3389\/fneur.2018.00552","article-title":"KL1333, a novel NAD(+) modulator, improves energy metabolism and mitochondrial dysfunction in MELAS fibroblasts","volume":"9","author":"Seo","year":"2018","journal-title":"Front. Neurol."},{"issue":"1","key":"10.1016\/j.cca.2026.121172_bb0050","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.bbadis.2016.10.028","article-title":"The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model","volume":"1863","author":"Frey","year":"2017","journal-title":"Biochim. Biophys. Acta Mol. basis Dis."},{"issue":"2","key":"10.1016\/j.cca.2026.121172_bb0055","doi-asserted-by":"crossref","DOI":"10.1172\/JCI136055","article-title":"Circulating markers of NADH-reductive stress correlate with mitochondrial disease severity","volume":"131","author":"Sharma","year":"2021","journal-title":"J. Clin. Invest."},{"issue":"1","key":"10.1016\/j.cca.2026.121172_bb0060","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1093\/brain\/awae308","article-title":"Optimizing rare disorder trials: a phase 1a\/1b randomized study of KL1333 in adults with mitochondrial disease","volume":"148","author":"Pizzamiglio","year":"2025","journal-title":"Brain"},{"issue":"2","key":"10.1016\/j.cca.2026.121172_bb0065","first-page":"C615","article-title":"Mitochondrial function in vivo evaluated by NADH fluorescence: from animal models to human studies","volume":"292","author":"Mayevsky","year":"2007","journal-title":"Am. J. Phys. Cell Phys."},{"issue":"6292","key":"10.1016\/j.cca.2026.121172_bb0070","doi-asserted-by":"crossref","first-page":"1474","DOI":"10.1126\/science.aad5168","article-title":"Biosensor reveals multiple sources for mitochondrial NAD+","volume":"352","author":"Cambronne","year":"2016","journal-title":"Science"},{"issue":"6282","key":"10.1016\/j.cca.2026.121172_bb0075","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1126\/science.aad4017","article-title":"Complementation of mitochondrial electron transport chain by manipulation of the NAD+\/NADH ratio","volume":"352","author":"Titov","year":"2016","journal-title":"Science"},{"key":"10.1016\/j.cca.2026.121172_bb0080","doi-asserted-by":"crossref","DOI":"10.1016\/j.molmet.2021.101269","article-title":"Diminished ketone interconversion, hepatic TCA cycle flux, and glucose production in D-\u03b2-hydroxybutyrate dehydrogenase hepatocyte-deficient mice","volume":"53","author":"Stagg","year":"2021","journal-title":"Mol. Metab."},{"key":"10.1016\/j.cca.2026.121172_bb0085","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.freeradbiomed.2025.03.029","article-title":"Reductive stress and mitochondrial dysfunction: the hidden link in chronic disease","volume":"233","author":"Mercola","year":"2025","journal-title":"Free Radic. Biol. Med."},{"issue":"7814","key":"10.1016\/j.cca.2026.121172_bb0090","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1038\/s41586-020-2337-2","article-title":"Hepatic NADH reductive stress underlies common variation in metabolic traits","volume":"583","author":"Goodman","year":"2020","journal-title":"Nature"},{"issue":"7506","key":"10.1016\/j.cca.2026.121172_bb0095","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1038\/nature13270","article-title":"Metformin suppresses gluconeogenesis by inhibiting mitochondrial glycerophosphate dehydrogenase","volume":"510","author":"Madiraju","year":"2014","journal-title":"Nature"},{"issue":"17","key":"10.1016\/j.cca.2026.121172_bb0100","doi-asserted-by":"crossref","DOI":"10.3390\/nu14173605","article-title":"Ketogenic diet treatment of defects in the mitochondrial malate aspartate shuttle and pyruvate carrier","volume":"14","author":"B\u00f6lsterli","year":"2022","journal-title":"Nutrients"},{"issue":"4","key":"10.1016\/j.cca.2026.121172_bb0105","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1016\/j.cmet.2020.02.017","article-title":"Serine catabolism feeds NADH when respiration is impaired","volume":"31","author":"Yang","year":"2020","journal-title":"Cell Metab."},{"key":"10.1016\/j.cca.2026.121172_bb0110","doi-asserted-by":"crossref","DOI":"10.1016\/j.talanta.2020.122048","article-title":"Determination of ketone bodies in biological samples via rapid UPLC-MS\/MS","volume":"225","author":"Puchalska","year":"2021","journal-title":"Talanta"},{"issue":"46","key":"10.1016\/j.cca.2026.121172_bb0115","doi-asserted-by":"crossref","first-page":"16976","DOI":"10.1021\/acs.analchem.3c03277","article-title":"Diazo-carboxyl click derivatization enables sensitive analysis of carboxylic acid metabolites in biosamples","volume":"95","author":"Li","year":"2023","journal-title":"Anal. Chem."},{"key":"10.1016\/j.cca.2026.121172_bb0120","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.mito.2019.02.001","article-title":"Biomarkers and clinical rating scales for sodium pyruvate therapy in patients with mitochondrial disease","volume":"48","author":"Koga","year":"2019","journal-title":"Mitochondrion"},{"issue":"9","key":"10.1016\/j.cca.2026.121172_bb0125","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1016\/S1474-4422(11)70155-7","article-title":"FGF-21 as a biomarker for muscle-manifesting mitochondrial respiratory chain deficiencies: a diagnostic study","volume":"10","author":"Suomalainen","year":"2011","journal-title":"Lancet Neurol."},{"issue":"5","key":"10.1016\/j.cca.2026.121172_bb0130","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1002\/ana.24506","article-title":"Growth differentiation factor 15 as a useful biomarker for mitochondrial disorders","volume":"78","author":"Yatsuga","year":"2015","journal-title":"Ann. Neurol."},{"issue":"1","key":"10.1016\/j.cca.2026.121172_bb0135","doi-asserted-by":"crossref","first-page":"2001","DOI":"10.1038\/s41598-021-81721-7","article-title":"Neurofilament light is a novel biomarker for mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes","volume":"11","author":"Zheng","year":"2021","journal-title":"Sci. Rep."},{"issue":"9","key":"10.1016\/j.cca.2026.121172_bb0140","doi-asserted-by":"crossref","first-page":"4985","DOI":"10.1007\/s00415-022-11146-3","article-title":"Circulating cell-free mtDNA release is associated with the activation of cGAS-STING pathway and inflammation in mitochondrial diseases","volume":"269","author":"Zhao","year":"2022","journal-title":"J. Neurol."},{"issue":"6","key":"10.1016\/j.cca.2026.121172_bb0145","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1016\/j.cmet.2019.08.019","article-title":"Fibroblast growth factor 21 drives dynamics of local and systemic stress responses in mitochondrial myopathy with mtDNA deletions","volume":"30","author":"Forsstr\u00f6m","year":"2019","journal-title":"Cell Metab."},{"issue":"6","key":"10.1016\/j.cca.2026.121172_bb0150","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1002\/ana.24954","article-title":"Serum neurofilament light: a biomarker of neuronal damage in multiple sclerosis","volume":"81","author":"Disanto","year":"2017","journal-title":"Ann. Neurol."},{"issue":"5","key":"10.1016\/j.cca.2026.121172_bb0155","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1001\/jamaneurol.2016.6117","article-title":"Association of Plasma Neurofilament Light with Neurodegeneration in patients with Alzheimer disease","volume":"74","author":"Mattsson","year":"2017","journal-title":"JAMA Neurol."},{"issue":"19","key":"10.1016\/j.cca.2026.121172_bb0160","doi-asserted-by":"crossref","first-page":"1788","DOI":"10.1212\/WNL.0000000000003912","article-title":"Serum neurofilament light as a biomarker for mild traumatic brain injury in contact sports","volume":"88","author":"Shahim","year":"2017","journal-title":"Neurology"},{"issue":"11","key":"10.1016\/j.cca.2026.121172_bb0165","doi-asserted-by":"crossref","first-page":"e1104","DOI":"10.1212\/WNL.0000000000008088","article-title":"Blood NfL: a biomarker for disease severity and progression in Parkinson disease","volume":"93","author":"Lin","year":"2019","journal-title":"Neurology"},{"issue":"10","key":"10.1016\/j.cca.2026.121172_bb0170","doi-asserted-by":"crossref","first-page":"3041","DOI":"10.1093\/brain\/awaa246","article-title":"Mitochondrial damage-associated inflammation highlights biomarkers in PRKN\/PINK1 parkinsonism","volume":"143","author":"Borsche","year":"2020","journal-title":"Brain"},{"issue":"1","key":"10.1016\/j.cca.2026.121172_bb0175","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.ymgme.2007.11.018","article-title":"The in-depth evaluation of suspected mitochondrial disease","volume":"94","author":"Haas","year":"2008","journal-title":"Mol. Genet. Metab."},{"issue":"4","key":"10.1016\/j.cca.2026.121172_bb0180","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1002\/jimd.12402","article-title":"Inborn disorders of the malate aspartate shuttle","volume":"44","author":"Broeks","year":"2021","journal-title":"J. Inherit. Metab. Dis."},{"issue":"6","key":"10.1016\/j.cca.2026.121172_bb0185","doi-asserted-by":"crossref","first-page":"988","DOI":"10.2337\/dc15-2752","article-title":"\u03b1-Hydroxybutyric acid is a selective metabolite biomarker of impaired glucose tolerance","volume":"39","author":"Cobb","year":"2016","journal-title":"Diabetes Care"},{"issue":"5","key":"10.1016\/j.cca.2026.121172_bb0190","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0010883","article-title":"Alpha-hydroxybutyrate is an early biomarker of insulin resistance and glucose intolerance in a nondiabetic population","volume":"5","author":"Gall","year":"2010","journal-title":"PLoS One"},{"issue":"5","key":"10.1016\/j.cca.2026.121172_bb0195","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1016\/j.celrep.2015.09.054","article-title":"A metabolic signature of mitochondrial dysfunction revealed through a monogenic form of Leigh syndrome","volume":"13","author":"Thompson Legault","year":"2015","journal-title":"Cell Rep."},{"key":"10.1016\/j.cca.2026.121172_bb0200","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.aca.2016.08.025","article-title":"Rapid quantification of metabolic intermediates in blood by liquid chromatography-tandem mass spectrometry to investigate congenital lactic acidosis","volume":"942","author":"Peng","year":"2016","journal-title":"Anal. Chim. Acta"}],"container-title":["Clinica Chimica Acta"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009898126003542?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009898126003542?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T04:19:54Z","timestamp":1786421994000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0009898126003542"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":40,"alternative-id":["S0009898126003542"],"URL":"https:\/\/doi.org\/10.1016\/j.cca.2026.121172","relation":{},"ISSN":["0009-8981"],"issn-type":[{"value":"0009-8981","type":"print"}],"subject":[],"published":{"date-parts":[[2026,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Detection of NADH\/NAD+ dysregulation in MELAS via diazo-carboxyl click derivatization mass spectrometry","name":"articletitle","label":"Article Title"},{"value":"Clinica Chimica Acta","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.cca.2026.121172","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}],"article-number":"121172"}}