{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T01:23:31Z","timestamp":1790126611702,"version":"4.0.1"},"reference-count":34,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2009,6,7]],"date-time":"2009-06-07T00:00:00Z","timestamp":1244332800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100004807","name":"California Department of Fish and Game","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004807","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The content of coenzyme Q<jats:sub>10<\/jats:sub> (CoQ<jats:sub>10<\/jats:sub>) was examined in skin fibroblasts of 10 patients with mevalonic aciduria (MVA) and of 22 patients with methylmalonic aciduria (MMA). Patients with these inborn errors of metabolism are thought to be at risk for CoQ<jats:sub>10<\/jats:sub> depletion either by direct inhibition of the proximal pathway of CoQ<jats:sub>10<\/jats:sub> synthesis (MVA) or indirectly by inhibition of mitochondrial energy metabolism (MMA). We demonstrated that CoQ<jats:sub>10<\/jats:sub> concentrations were not significantly different from controls in MVA patients, suggesting that there may be upregulatory effects. On the other hand the CoQ<jats:sub>10<\/jats:sub> content in fibroblasts of patients with MMA was significantly reduced.<\/jats:p>","DOI":"10.1007\/s10545-009-1150-8","type":"journal-article","created":{"date-parts":[[2009,6,6]],"date-time":"2009-06-06T07:58:08Z","timestamp":1244275088000},"page":"570-575","source":"Crossref","is-referenced-by-count":30,"title":["Coenzyme Q<sub>10<\/sub> is decreased in fibroblasts of patients with methylmalonic aciduria but not in mevalonic aciduria"],"prefix":"10.1002","volume":"32","author":[{"given":"D.","family":"Haas","sequence":"first","affiliation":[{"name":"Department of General Pediatrics, Division of Inborn Metabolic Diseases University Children's Hospital Im Neuenheimer Feld 430 Heidelberg 69120 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Niklowitz","sequence":"additional","affiliation":[{"name":"Vestische Kinderklinik Datteln University Witten\/Herdecke Datteln Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"H\u00f6rster","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, Division of Inborn Metabolic Diseases University Children's Hospital Im Neuenheimer Feld 430 Heidelberg 69120 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. 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