RT Book, Section A1 Fowler, Brian A1 Blom, Henk A2 Sarafoglou, Kyriakie A2 Hoffmann, Georg F. A2 Roth, Karl S. SR Print(0) ID 1140316789 T1 Disorders of Transsulfuration T2 Pediatric Endocrinology and Inborn Errors of Metabolism, 2e YR 2017 FD 2017 PB McGraw-Hill Education PP New York, NY SN 9780071773140 LK accesspediatrics.mhmedical.com/content.aspx?aid=1140316789 RD 2024/12/08 AB Transsulfuration is the major metabolic route for the catabolism of methionine in humans and involves the formation of S-adenosylmethionine (AdoMet), S-adenosylhomocysteine (AdoHcy), homocysteine, cystathionine, and cysteine.1,2,3 The methionine transamination pathway is also involved in methionine metabolism but is a minor pathway that occurs only when methionine levels are abnormally high (>350 μmol/L),2,3 and leads to the formation of 4-methylthio-2-oxobutyrate, 3-methylthiopropionate, methanethiol and dimethylsulphide.4