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Inborn errors of sulfur-containing amino acid metabolism Finkelstein JDJ Nutr 2006[Jun]; 136 (6 Suppl): 1750S-1754STwo superimposed metabolic sequences, transsulfuration and the methionine/homocysteine cycle, form the pathway for methionine metabolism in mammalian liver. This combined pathway was formulated first to explain observations in subjects with homocystinuria caused by cystathionine synthase deficiency. Since that time additional inborn errors have been discovered, and currently we know of human subjects with isolated defects in all of the reactions of the combined pathway with only one exception: betaine homocysteine methyltransferase. Studies of these inborn errors have contributed significantly to our knowledge of human methionine metabolism and to the clinical consequences of impaired metabolism. Transsulfuration appears to function primarily for the metabolism of excess methionine, and each of the 5 defects in this pathway results in the accumulation of 1 or more of the normal metabolites. Thus, studies of these disorders may provide insight into both the potential pathological sequelae of nutritional methionine excess as well as whether laboratory testing allows the detection of excess.|*Amino Acid Metabolism, Inborn Errors[MESH]|Adenosylhomocysteinase/deficiency[MESH]|Amino Acids, Sulfur/*metabolism[MESH]|Betaine-Homocysteine S-Methyltransferase/metabolism[MESH]|Cystathionine gamma-Lyase/deficiency[MESH]|Glycine N-Methyltransferase/deficiency[MESH]|Humans[MESH]|Kidney/metabolism[MESH]|Liver/metabolism[MESH]|Methionine Adenosyltransferase/deficiency[MESH]|Methionine/metabolism[MESH]|Sulfur/metabolism[MESH] |
