TY - CHAP M1 - Book, Section TI - Disorders of Transsulfuration A1 - Fowler, Brian A1 - Blom, Henk A2 - Sarafoglou, Kyriakie A2 - Hoffmann, Georg F. A2 - Roth, Karl S. PY - 2017 T2 - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e 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 SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/10 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1140316789 ER -