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 (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)).
For a description of pathway objects, see the WikiPathways Legend.
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
Pathway Ontology
sulfite oxidase deficiency pathway homocystinuria pathway cysteine and methionine metabolic pathway methionine degradation pathway altered metal homeostasis pathway cystathioninuria pathway disease pathway hypermethioninemia pathway hypermethioninemia pathway glycine N-methyltransferase deficiency pathwayDisease Ontology
hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase hypermethioninemia isolated sulfite oxidase deficiency homocystinuria cystathioninuria hypermethioninemia due to adenosine kinase deficiency glycine N-methyltransferase deficiencyLabel | 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 | |
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 | |
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