{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T20:36:57Z","timestamp":1790800617953,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1999,4,1]],"date-time":"1999-04-01T00:00:00Z","timestamp":922924800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1999,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Maple syrup urine disease is an autosomal recessive inherited disorder of branched\u2010chain amino acid metabolism due to deficiency of the branched\u2010chain \u03b1\u2010keto acid dehydrogenase complex. The disease was originally named after the characteristic sweet aroma, reminiscent of maple syrup, present in the body fluids of affected patients. Until now, the substance responsible for the odour has not been positively identified. Using enantioselective multidimensional gas chromatography\u2010mass spectrometry (enantio\u2010MDGC\u2010MS), we could demonstrate that 4,5\u2010dimethyl\u20103\u2010hydroxy\u20102[5H]\u2010furanone (sotolone), a well\u2010known flavour impact compound present in fenugreek and lovage, was present in urine from seven patients with maple syrup urine disease. Urine samples from healthy control persons lacked sotolone. We have shown that sotolone is responsible for the characteristic odour of maple syrup urine disease and, since maple syrup also contains sotolone, the naming of this disease appears to be correct.<\/jats:p>","DOI":"10.1023\/a:1005433516026","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T22:25:23Z","timestamp":1040509523000},"page":"107-114","source":"Crossref","is-referenced-by-count":67,"title":["4,5\u2010Dimethyl\u20103\u2010hydroxy\u20102[5H]\u2010furanone (sotolone) \u2014 The odour of maple syrup urine disease"],"prefix":"10.1002","volume":"22","author":[{"given":"F.","family":"Podebrad","sequence":"first","affiliation":[{"name":"Institute of Food Chemistry University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Heil","sequence":"additional","affiliation":[{"name":"Institute of Food Chemistry University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Reichert","sequence":"additional","affiliation":[{"name":"Institute of Food Chemistry University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Mosandl","sequence":"additional","affiliation":[{"name":"Institute of Food Chemistry University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. C.","family":"Sewell","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"B\u00f6hles","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Frankfurt  Frankfurt am Main Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1999,4]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0031-9422(00)97870-1"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198108203050823"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1002\/9783527612390","volume-title":"Common Fragrance and Flavor Materials: Preparation Properties and Uses","author":"Bauer K","year":"1997"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1745-459X.1991.tb00506.x"},{"key":"e_1_2_1_6_1","article-title":"Stereodifferenzierung und herkunftsspezi\u00d0sche Analyse aromarelevanter Dihydrofuranone. 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