Methionine metabolism leading to sulfur amino acids and related disorders (WP4292)

This pathway visualises the conversion of methionine to inorganic sulphates (involving the formation of homocysteine, a toxic intermediate also related to MTHFR deficiency []). 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)).
last edited


HRitter, Egonw, Khanspers, DeSl, Elisson nl, IreneHemel, MaintBot, Fehrhart, Eweitz, and Finterly

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Pathway Ontology: hypermethioninemia pathway homocystinuria pathway disease pathway glycine N-methyltransferase deficiency pathway hypermethioninemia pathway cystathioninuria pathway altered metal homeostasis pathway methionine degradation pathway sulfite oxidase deficiency pathway cysteine and methionine metabolic pathway

Disease Ontology: glycine N-methyltransferase deficiency homocystinuria hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase hypermethioninemia isolated sulfite oxidase deficiency cystathioninuria hypermethioninemia due to adenosine kinase deficiency


Label Type Compact Identifier
Methionine Metabolite chebi:16643
Sulphocysteine Metabolite chebi:27891
Cystathionine Metabolite chebi:17482
ADKD GeneProduct ensembl:ENSG00000156110
Sulfate Metabolite hmdb:HMDB0001448
Taurine Metabolite chebi:15891
Sulfite Metabolite chebi:26823
S-Adenosylmethionine Metabolite chebi:67040
Cysteine Metabolite chebi:15356
CSAT Protein eccode:2.6.1.-
Methionine adenosyltransferase I/III Protein eccode:
X-MT Protein None
Vit. B12pathway Pathway wikipathways:WP4271
CyD Protein uniprot:Q16878
CySD Protein uniprot:Q9Y600
SUOX GeneProduct eccode:
HTOx Protein eccode:
Adenosine Metabolite chebi:16335
S-Adenosylhomocysteine Metabolite chebi:16680
Methyl-cobalamin Metabolite chebi:28115
MS Protein uniprot:Q99707
Hypotaurine Metabolite chebi:16668
Build proteins Pathway wikipathways:WP3580
Glycine Metabolite hmdb:HMDB0000123
BMT Protein uniprot:E5RJH0
Betaine Metabolite chebi:17750
MAT2A Protein uniprot:B4DEX8
AHCY Protein uniprot:P23526
Cysteine sulfinic acid Metabolite chebi:61085
ATP Metabolite chebi:15422
CBS GeneProduct ensembl:ENSG00000160200
AMP Metabolite chebi:16027
MAT1A Protein uniprot:Q00266
Sarcosine Metabolite chebi:15611
Diphosphate ion Metabolite wikidata:Q290828
GNMT Protein uniprot:Q14749
Beta-Sulfinyl pyruvate Metabolite hmdb:HMDB0002332
Homocysteine Metabolite chebi:17230
Phosphate ion Metabolite wikidata:Q177811
Methylation onDNA, RNA, hormones,Lipids, proteins, Creatine-P etc. Pathway wikipathways:WP704
Methyl-cobalamin Metabolite chebi:28115
MS Protein uniprot:Q99707
CTH GeneProduct ensembl:ENSG00000116761
MAT2B Protein uniprot:H7C0X7
H2O Metabolite chebi:15377
Vit. B12pathway Pathway wikipathways:WP4271


  1. Griffith OW. Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate. J Biol Chem. 1983 Feb 10;258(3):1591–8. PubMed Europe PMC Scholia
  2. Linnebank M, Lagler F, Muntau AC, Röschinger W, Olgemöller B, Fowler B, et al. Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. J Inherit Metab Dis. 2005;28(6):1167–8. PubMed Europe PMC Scholia
  3. PubMed Europe PMC Scholia