{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T15:22:13Z","timestamp":1747149733544,"version":"3.40.5"},"reference-count":24,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,7,14]],"date-time":"2023-07-14T00:00:00Z","timestamp":1689292800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100020225","name":"Universidade de Fortaleza","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100020225","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The branched\u2010chain amino acids (BCAA) leucine, valine, and isoleucine provide precursors for monomethyl branched\u2010chain fatty acids (BCFA). Established reference ranges for BCFAs are lacking. In maple syrup urine disease (MSUD), a rare inborn error of BCAA metabolism, the endogen production is impaired and MSUD patients are treated with a low protein (low BCAA) diet. The protein restriction may affect the dietary intake of BCFA, depending on the dietary choices made. Patients with MSUD are prescribed a more or less protein\u2010restricted diet depending on the severity of the disease. The combination of a protein\u2010restricted diet and subsequent impaired endogenous synthesis may render MSUD patients sensitive to BCFA deficiency, with yet unknown implications. To investigate the possibility of lower circulatory BCFA levels in MSUD that favors dietary BCFA supplementation, we first established fasting\u2010state reference ranges for selected BCFAs and saturated\/unsaturated fatty acids in plasma. Then, the effect of fasting on BCFA levels was evaluated by comparing the distribution in a fasting versus a non\u2010fasting cohort. To test the hypothesis that BCFA deficiency could contribute to MSUD pathophysiology, we recruited patients with intermittent, intermediate, and classical form of MSUD and analyzed the corresponding BCFA z\u2010scores. None of the BCFA species had |z\u2010scores|\u2009&gt;\u20092 relative to the reference range. Our findings do not support the requirement of BCFA supplementation in MSUD patients. The origin of BCFAs is discussed. Impaired capacity to synthesize BCFA do not manifest as reduced plasma levels in MSUD, suggesting that endogenous synthesis is dispensable for plasma levels.<\/jats:p>","DOI":"10.1002\/jmd2.12380","type":"journal-article","created":{"date-parts":[[2023,7,15]],"date-time":"2023-07-15T04:39:00Z","timestamp":1689395940000},"page":"360-366","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Fasting and non\u2010fasting plasma levels of monomethyl branched chain fatty acids: Implications for maple syrup urine disease"],"prefix":"10.1002","volume":"64","author":[{"given":"Trine","family":"Tangeraas","sequence":"first","affiliation":[{"name":"Department of Newborn Screening Oslo University Hospital  Oslo Norway"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erle","family":"Kristensen","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry Oslo University Hospital  Oslo 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