{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:04:52Z","timestamp":1791327892927,"version":"4.3.1"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1984,5,3]],"date-time":"1984-05-03T00:00:00Z","timestamp":452390400000},"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":[[1985,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    A new metabolic disorder characterised by the excessive excretion of \u03b2\u2010alanine, 3\u2010hydroxypropionic acid,\n                    <jats:italic>R<\/jats:italic>\n                    \u2010and\n                    <jats:italic>S<\/jats:italic>\n                    \u20103\u2010amino\u2010 and 3\u2010hydroxyisobutyric acids and\n                    <jats:italic>S<\/jats:italic>\n                    \u20102\u2010(hydroxymethyl)butyric acid is probably due to deficient activities of malonic, methylmalonic and ethylmalonic semialdehyde dehydrogenases. These dehydrogenation reactions could be mediated by one enzyme, or by enzymes with a common subunit, and both\n                    <jats:italic>R<\/jats:italic>\n                    \u2010 and\n                    <jats:italic>S<\/jats:italic>\n                    \u2010methylmalonic semialdehydes seem to be equally affected. The patient is now aged 4 years and has developed normally. He has a persistent gross hypermethioninaemia which is probably unrelated to the other biochemical abnormalities.\n                  <\/jats:p>","DOI":"10.1007\/bf01801669","type":"journal-article","created":{"date-parts":[[2005,6,27]],"date-time":"2005-06-27T19:55:39Z","timestamp":1119902139000},"page":"75-79","source":"Crossref","is-referenced-by-count":59,"title":["Excessive excretion of \u03b2\u2010alanine and of 3\u2010hydroxypropionic,\n                    <i>R<\/i>\n                    \u2010 and\n                    <i>S<\/i>\n                    \u20103\u2010aminoisobutyric,\n                    <i>R<\/i>\n                    \u2010 and\n                    <i>S<\/i>\n                    \u20103\u2010hydroxyisobutyric and\n                    <i>S<\/i>\n                    \u20102\u2010(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes"],"prefix":"10.1002","volume":"8","author":[{"given":"R. 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Box 134 Sheffield S6 1TP UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Green","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Birmingham Children's Hospital, Ladywood Middleway  Birmingham B16 8ET UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Smith","sequence":"additional","affiliation":[{"name":"Biochemistry Department Bradford Royal Infirmary, Duckworth Lane  Bradford BD9 6RJ UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1984,5,3]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)77166-X"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)34704-X"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)50783-3"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02263600"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410090503"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(81)90045-0"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/bit.260130204"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0304-4165(68)90267-5"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-4347(00)81817-4"},{"key":"e_1_2_1_11_1","first-page":"69","article-title":"Methylmalonic semialdehyde","volume":"7","author":"Kupiecki F. 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