Methionine metabolism leading to sulfur amino acids and related disorders (WP4292)
Homo sapiens
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)).
Authors
Hermann Ritter , Egon Willighagen , Kristina Hanspers , Denise Slenter , Elisson nl , Irene Hemel , Friederike Ehrhart , Eric Weitz , and Finterly HuActivity
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Organisms
Homo sapiensCommunities
Inherited Metabolic Disorders (IMD) Pathways Rare DiseasesAnnotations
Disease Ontology
hypermethioninemia due to adenosine kinase deficiency homocystinuria hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase hypermethioninemia glycine N-methyltransferase deficiency cystathioninuria isolated sulfite oxidase deficiencyPathway Ontology
cystathioninuria pathway hypermethioninemia pathway glycine N-methyltransferase deficiency pathway cysteine and methionine metabolic pathway disease pathway homocystinuria pathway sulfite oxidase deficiency pathway altered metal homeostasis pathway methionine degradation pathway hypermethioninemia pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| Methionine | Metabolite | chebi:16643 | |
| Sulphocysteine | Metabolite | chebi:27891 | |
| Cystathionine | Metabolite | chebi:17482 | |
| Sulfate | Metabolite | hmdb:HMDB0001448 | |
| Taurine | Metabolite | chebi:15891 | |
| Sulfite | Metabolite | chebi:26823 | |
| S-Adenosylmethionine | Metabolite | chebi:67040 | |
| Cysteine | Metabolite | chebi:15356 | |
| Adenosine | Metabolite | chebi:16335 | |
| S-Adenosylhomocysteine | Metabolite | chebi:16680 | |
| Methyl-cobalamin | Metabolite | chebi:28115 | '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 |
| Hypotaurine | Metabolite | chebi:16668 | |
| Glycine | Metabolite | hmdb:HMDB0000123 | |
| Betaine | Metabolite | chebi:17750 | |
| Cysteine sulfinic acid | Metabolite | chebi:61085 | aka 3-sulfino-L-alanine |
| ATP | Metabolite | chebi:15422 | |
| AMP | Metabolite | chebi:16027 | |
| Sarcosine | Metabolite | chebi:15611 | |
| Diphosphate ion | Metabolite | wikidata:Q290828 | |
| Beta-sulfinyl pyruvate | Metabolite | hmdb:HMDB0002332 | |
| Homocysteine | Metabolite | chebi:17230 | |
| Phosphate ion | Metabolite | wikidata:Q177811 | |
| Methyl-cobalamin | Metabolite | chebi:28115 | '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 |
| H2O | Metabolite | chebi:15377 | |
| ADKD | GeneProduct | ensembl:ENSG00000156110 | aka Adenosine kinase |
| SUOX | GeneProduct | eccode:1.8.3.1 | Sulfite oxidase |
| CBS | GeneProduct | ensembl:ENSG00000160200 | cystathioniune beta-synthase |
| CTH | GeneProduct | ensembl:ENSG00000116761 | cystathioniune gamma-lyase |
| CSAT | Protein | eccode:2.6.1.- | cysteine sulphinate alpha-oxoglutarate aminotransferaseNot available in Uniprot (yet) 2021-01-17 |
| Methionine adenosyltransferase I/III | Protein | eccode:2.5.1.6 | |
| CyD | Protein | uniprot:Q16878 | Cysteine dioxygenase type 1 |
| CySD | Protein | uniprot:Q9Y600 | Cysteine sulfinic acid decarboxylase |
| HTOx | Protein | eccode:1.8.1.3 | hypothaurine:NAD+ oxidoreductaseTwo known cofactors: heme,Molybdenum |
| MS | Protein | uniprot:Q99707 | 5-methyltetrahydrofolate-homocysteine methyltransferase |
| BMT | Protein | uniprot:E5RJH0 | aka Betaine--homocysteine S-methyltransferase 1 |
| MAT2A | Protein | uniprot:B4DEX8 | |
| AHCY | Protein | uniprot:P23526 | S-adenosylhomocysteine hydrolase |
| MAT1A | Protein | uniprot:Q00266 | |
| GNMT | Protein | uniprot:Q14749 | |
| MS | Protein | uniprot:Q99707 | 5-methyltetrahydrofolate-homocysteine methyltransferase |
| MAT2B | Protein | uniprot:H7C0X7 |
References
- Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Springer; 2014. 0 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
- Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate. Griffith OW. J Biol Chem. 1983 Feb 10;258(3):1591–8. PubMed Europe PMC Scholia
- Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. Linnebank M, Lagler F, Muntau AC, Röschinger W, Olgemöller B, Fowler B, et al. J Inherit Metab Dis. 2005;28(6):1167–8. PubMed Europe PMC Scholia