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 [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)).
last edited

Authors

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

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organism

Homo sapiens

Communities

Inborn Errors of Metabolism (IEM) Pathways Rare Diseases

Annotations

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

Participants

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:2.5.1.6
X-MT Protein None
Vit. B12pathway Pathway wikipathways:WP4271
CyD Protein uniprot:Q16878
CySD Protein uniprot:Q9Y600
SUOX GeneProduct eccode:1.8.3.1
HTOx Protein eccode:1.8.1.3
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

References

  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