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Interest has been stimulated by the discovery of two novel disorders, glycine <jats:italic>N<\/jats:italic>\u2010methyltransferase deficiency and <jats:italic>S<\/jats:italic>\u2010adenosylhomocysteine hydrolase deficiency. Another disorder in this pathway, methionine adenosyltransferase deficiency, has been increasingly detected, thanks to the expansion of newborn screening programmes by tandem mass spectrometry technology. These significant steps allow important insight into the pathogenesis of these three disorders, as well as into the mechanisms of damage to various organs (liver, brain, muscle) and point to the relevance of these disorders for crucial biological processes such as methylation, transsulfuration or carcinogenesis in mammals, the pathogenesis of numerous pathological conditions, in particular those associated with hyperhomocysteinaemia, the action and possible toxicity of some drugs or consequences of nutritional variations. This review summarizes current knowledge of three inherited disorders in this metabolic pathway and draws attention to their much broader significance for human health and understanding of important biological processes.<\/jats:p>","DOI":"10.1007\/s10545-009-1146-4","type":"journal-article","created":{"date-parts":[[2009,7,7]],"date-time":"2009-07-07T11:50:12Z","timestamp":1246967412000},"page":"459-471","source":"Crossref","is-referenced-by-count":49,"title":["Inherited disorders in the conversion of methionine to homocysteine"],"prefix":"10.1002","volume":"32","author":[{"given":"Ivo","family":"Bari\u0107","sequence":"first","affiliation":[{"name":"Department of Pediatrics University Hospital Center and School of Medicine Zagreb Croatia"},{"name":"Department of Pediatrics University Hospital Center Ki\u0161pati\u0107eva 12, Rebro Zagreb 10000 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