{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T20:12:18Z","timestamp":1767211938422,"version":"build-2065373602"},"reference-count":39,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2013,4,10]],"date-time":"2013-04-10T00:00:00Z","timestamp":1365552000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rapid Comm Mass Spectrometry"],"published-print":{"date-parts":[[2013,5,15]]},"abstract":"\n RATIONALE<\/jats:title>\n \n Neurological dysfunction is common in primary coenzyme Q\n 10<\/jats:sub>\n (2,3\u2010dimethoxy, 5\u2010methyl, 6\u2010polyisoprene parabenzoquinone; CoQ\n 10<\/jats:sub>\n ; ubiquinone) deficiencies, the most readily treatable subgroup of mitochondrial disorders. Therapeutic benefit from CoQ\n 10<\/jats:sub>\n supplementation has also been noted in other neurodegenerative diseases. CoQ\n 10<\/jats:sub>\n can be measured by high\u2010performance liquid chromatography (HPLC) in plasma, muscle or leucocytes; however, there is no reliable method to quantify CoQ\n 10<\/jats:sub>\n in cerebrospinal fluid (CSF). Additionally, many methods use CoQ\n 9<\/jats:sub>\n , an endogenous ubiquinone in humans, as an internal standard.\n <\/jats:p>\n <\/jats:sec>\n \n METHODS<\/jats:title>\n \n Deuterated CoQ\n 10<\/jats:sub>\n (\n \n d\n 6<\/jats:sub>\n <\/jats:italic>\n \u2010CoQ\n 10<\/jats:sub>\n ) was synthesised by a novel, simple, method. Total CoQ\n 10<\/jats:sub>\n was measured by liquid chromatography\/tandem mass spectrometry (LC\/MS\/MS) using\n \n d\n 6<\/jats:sub>\n <\/jats:italic>\n \u2010CoQ\n 10<\/jats:sub>\n as internal standard and 5\u2009mM methylamine as an ion\u2010pairing reagent. Chromatography was performed using a Hypsersil GOLD C4 column (150\u2009\u00d7\u20093\u2009mm, 3\u2009\u00b5m).\n <\/jats:p>\n <\/jats:sec>\n \n RESULTS<\/jats:title>\n \n CoQ\n 10<\/jats:sub>\n levels were linear over a concentration range of 0\u2013200 nM (\n R<\/jats:italic>\n 2<\/jats:sup>\n \u2009=\u20090.9995). The lower limit of detection was 2 nM. The inter\u2010assay coefficient of variation (CV) was 3.6% (10 nM) and 4.3% (20 nM), and intra\u2010assay CV 3.4% (10 nM) and 3.6% (20 nM). Reference ranges were established for CoQ\n 10<\/jats:sub>\n in CSF (5.7\u20138.7 nM;\n n<\/jats:italic>\n \u2009=\u200917), fibroblasts (57.0\u2013121.6 pmol\/mg;\n n<\/jats:italic>\n = 50) and muscle (187.3\u2013430.1 pmol\/mg;\n n<\/jats:italic>\n \u2009=\u200915).\n <\/jats:p>\n <\/jats:sec>\n \n CONCLUSIONS<\/jats:title>\n \n Use of\n \n d\n 6<\/jats:sub>\n <\/jats:italic>\n \u2010CoQ\n 10<\/jats:sub>\n internal standard has enabled the development of a sensitive LC\/MS\/MS method to accurately determine total CoQ\n 10<\/jats:sub>\n levels. Clinical applications of CSF CoQ\n 10<\/jats:sub>\n determination include identification of patients with cerebral CoQ\n 10<\/jats:sub>\n deficiency, and monitoring CSF CoQ\n 10<\/jats:sub>\n levels following supplementation. Copyright \u00a9 2013 John Wiley & Sons, Ltd.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/rcm.6529","type":"journal-article","created":{"date-parts":[[2013,4,14]],"date-time":"2013-04-14T23:11:50Z","timestamp":1365981110000},"page":"924-930","source":"Crossref","is-referenced-by-count":20,"title":["Coenzyme Q\n 10<\/sub>\n quantification in muscle, fibroblasts and cerebrospinal fluid by liquid chromatography\/tandem mass spectrometry using a novel deuterated internal standard"],"prefix":"10.1002","volume":"27","author":[{"given":"Kate E. C.","family":"Duberley","sequence":"first","affiliation":[{"name":"Department of Molecular Neuroscience, UCL Institute of Neurology Queen Square London WC1N 3BG UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iain P.","family":"Hargreaves","sequence":"additional","affiliation":[{"name":"Neurometabolic Unit, National Hospital of Neurology and Neurosurgery, Queen Square London WC1N 3BG UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Korn\u2010Anong","family":"Chaiwatanasirikul","sequence":"additional","affiliation":[{"name":"Unit of Paediatric Surgery, UCL Institute of Child Health London WC1N 1EH UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon J. 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