This pathway visualises the conversion of methionine to inorganic sulphates (involving the formation of homocysteine, a toxic intermediate also related to MTHFR deficiency ([WP4288](https://www.wikipathways.org/index.php/Pathway:WP4288)). Methionine, an essential amino acid, is taken in from diet and can be created from breaking down proteins. This pathway was inspired by Chapter 3 of the book of Blau (ISBN 3642403360 (978-3642403361)). c47 aka Adenosine kinase cysteine sulphinate alpha-oxoglutarate aminotransferase Not available in Uniprot (yet) 2021-01-17 S-adenosylmethionine-dependent transmethylation enzymes Cysteine dioxygenase type 1 Cysteine sulfinic acid decarboxylase Sulfite oxidase hypothaurine:NAD+ oxidoreductase Two known cofactors: heme,Molybdenum "This vitamer is one of two active coenzymes used by vitamin B12-dependent enzymes and is the specific vitamin B12 form used by 5-methyltetrahydrofolate-homocysteine methyltransferase (MTR), also known as methionine synthase" https://en.wikipedia.org/wiki/Methylcobalamin 5-methyltetrahydrofolate-homocysteine methyltransferase aka Betaine--homocysteine S-methyltransferase 1 S-adenosylhomocysteine hydrolase aka 3-sulfino-L-alanine cystathioniune beta-synthase "This vitamer is one of two active coenzymes used by vitamin B12-dependent enzymes and is the specific vitamin B12 form used by 5-methyltetrahydrofolate-homocysteine methyltransferase (MTR), also known as methionine synthase" https://en.wikipedia.org/wiki/Methylcobalamin 5-methyltetrahydrofolate-homocysteine methyltransferase cystathioniune gamma-lyase Not available in Rhea (yet) 2021-01-17 f2b S-adenosylmethionine-dependent transmethylation reactions eac DISEASE DISEASE aka HYPERMETHIONINEMIA WITH S-ADENOSYLHOMOCYSTEINE HYDROLASE DEFICIENCY DISEASE aka hypermethioninemia due to adenosine kinase deficiency. DISEASE aka classical homocystinuria, CYSTATHIONINURIA DISEASE aka cystathionine gamma-lyase deficiency DISEASE aka isolated sulphite oxidase deficiency DISEASE aka HYPERMETHIONINEMIA 16435220 PubMed Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. J Inherit Metab Dis 2005 Linnebank M Lagler F Muntau AC Röschinger W Olgemöller B Fowler B Koch HG sulfite oxidase deficiency pathway PW:0001647 Pathway Ontology hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase DOID:0111039 Disease homocystinuria pathway PW:0001841 Pathway Ontology cysteine and methionine metabolic pathway PW:0001078 Pathway Ontology hypermethioninemia DOID:0050544 Disease methionine degradation pathway PW:0001302 Pathway Ontology isolated sulfite oxidase deficiency DOID:0111270 Disease altered metal homeostasis pathway PW:0001832 Pathway Ontology cystathioninuria pathway PW:0002561 Pathway Ontology homocystinuria DOID:9263 Disease disease pathway PW:0000013 Pathway Ontology hypermethioninemia pathway PW:0001868 Pathway Ontology 6822523 PubMed Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate. J Biol Chem 1983 Griffith OW 9783642403361 ISBN Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases Springer-Verlag Berlin Heidelberg 2014 9783642403361 PubMed Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases Springer-Verlag Berlin Heidelberg Ed.4 2014 Nenad Blau Marinus Duran K Michael Gibson Carlo Dionisi-Vici cystathioninuria DOID:0090142 Disease hypermethioninemia pathway PW:0002471 Pathway Ontology hypermethioninemia due to adenosine kinase deficiency DOID:0111038 Disease glycine N-methyltransferase deficiency pathway PW:0002299 Pathway Ontology glycine N-methyltransferase deficiency DOID:0111037 Disease