Cysteine and methionine catabolism (WP4504)
Homo sapiens
This pathway visualises the conversion of methionine to cysteine, after which it is further metabolised in either an oxidative or non-oxidative manner. Several of these metabolites are altered in people suffering from MoCD and SO; changes in metabolite concentrations (comparing MoCD cases to controls) are highlighted in blue (see [https://www.wikipathways.org/index.php/Pathway:WP4507] for the pathway on MoCD). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).
Authors
Denise Slenter , Egon Willighagen , Irene Hemel , Daniel Domingo-Fdez , Friederike Ehrhart , and Finterly HuActivity
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Organisms
Homo sapiensCommunities
Inherited Metabolic Disorders (IMD) Pathways Rare DiseasesAnnotations
Pathway Ontology
methionine degradation pathway cysteine and methionine metabolic pathway cysteine metabolic pathway methionine cycle/metabolic pathway disease pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| Methionine | Metabolite | chebi:57844 | |
| Tetra-hydrofolate | Metabolite | chebi:57453 | |
| Cystathionine | Metabolite | chebi:58161 | |
| -CH3 | Metabolite | chebi:32875 | |
| ATP | Metabolite | chebi:30616 | |
| Sulfate | Metabolite | chebi:16189 | |
| KG | Metabolite | chebi:16810 | aka a-ketoglutarate (a-KG) |
| Taurine | Metabolite | chebi:15891 | |
| 3-Mercaptopyruvic acid | Metabolite | chebi:57678 | |
| Sulfite | Metabolite | chebi:17359 | |
| S-Adenosylmethionine | Metabolite | chebi:59789 | |
| Cysteine | Metabolite | chebi:35235 | |
| CO2 | Metabolite | chebi:16526 | |
| SO3 2- | Metabolite | chebi:17359 | AKA sulfite ion. |
| Glutathione | Metabolite | chebi:57925 | |
| S-Adenosylhomocysteine | Metabolite | chebi:57856 | |
| Dimethylglycine | Metabolite | chebi:58251 | |
| 5-Methyltetrahydrofolate | Metabolite | chebi:18608 | |
| Hypotaurine | Metabolite | chebi:57853 | |
| gamma-Glutamylcysteine | Metabolite | chebi:58173 | |
| Cystine | Metabolite | chebi:17376 | |
| H2S | Metabolite | chebi:29919 | aka Hydrogen sulfide |
| H2O | Metabolite | chebi:15377 | |
| S-sulfocysteine | Metabolite | chebi:27891 | |
| Betaine | Metabolite | chebi:17750 | |
| alpha-KB | Metabolite | chebi:16763 | |
| Pyr | Metabolite | chebi:15361 | AKA Pyruvic acid |
| Cysteine sulfinic acid | Metabolite | chebi:61085 | |
| serine | Metabolite | chebi:33384 | |
| Glu | Metabolite | chebi:29985 | AKA glutamine |
| O2 | Metabolite | chebi:15379 | |
| thiosulfate(2−) | Metabolite | chebi:16094 | AKA thiosulfate ion |
| B-Sulfinyl pyruvate | Metabolite | hmdb:HMDB0002332 | |
| Homocysteine | Metabolite | chebi:58199 | |
| RSSH | Metabolite | chebi:30605 | AKA persulfurated thiol-containing compound (RSSH) |
| H2S | Metabolite | chebi:16136 | aka Hydrogen sulfide |
| Adenosine | Metabolite | chebi:16335 | |
| NH4+ | Metabolite | chebi:28938 | |
| KG | Metabolite | chebi:16810 | aka a-ketoglutarate (a-KG) |
| Glu | Metabolite | chebi:28300 | AKA glutamine |
| Glu | Metabolite | chebi:29985 | AKA glutamine |
| Gly | Metabolite | chebi:57305 | AKA glycine |
| MAT | GeneProduct | uniprot:Q00266 | aka Methionine adenosyltransferase |
| SO | GeneProduct | uniprot:P51687 | AKA Sulfite oxidase |
| MT | GeneProduct | brenda:2.1.1 | AKA Methyltransferase |
| SO | GeneProduct | brenda:1.8.3.1 | AKA Sulfite oxidase |
| GS | Protein | uniprot:P48637 | AKA Glutathione synthetase |
| MS | Protein | uniprot:Q99707 | AKA Methionine synthase |
| MPST | Protein | uniprot:P25325 | AKA 3-mercaptopyruvate sulfurtransferase |
| GCS | Protein | uniprot:Q14TF0 | AKA Glutamate-cysteine ligase |
| SAAH | Protein | uniprot:P23526 | AKA Adenosylhomocysteinase |
| CDO | Protein | uniprot:Q16878 | Cysteine dioxygenase type 1 |
| CSD | Protein | uniprot:Q9Y600 | AKA Cysteine sulfinic acid decarboxylase |
| CSE | Protein | uniprot:P35520 | aka Cystathionine beta-synthase |
| AAT | Protein | uniprot:A0A140VK69 | AKA Aspartate aminotransferase |
| BHMT | Protein | uniprot:Q93088 | AKA Betaine--homocysteine S-methyltransferase 1 |
| CSE | Protein | uniprot:P32929 | Cystathionine gamma-lyase |
| SQR | Protein | uniprot:Q9Y6N5 | AKA quinone oxidoreductase |
| AAT | Protein | uniprot:P17174 | AKA Aspartate aminotransferase |
| CSE | Protein | uniprot:P35520 | aka Cystathionine beta-synthase |
| CSE | Protein | uniprot:P32929 | Cystathionine gamma-lyase |
| SQR | Protein | uniprot:Q9Y6N5 | |
| SDO | Protein | uniprot:O95571 | AKA sulfur dioxygenase |
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
- Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase. Smith AE, Matthews RG. Biochemistry. 2000 Nov 14;39(45):13880–90. PubMed Europe PMC Scholia
- Domain alternation switches B(12)-dependent methionine synthase to the activation conformation. Bandarian V, Pattridge KA, Lennon BW, Huddler DP, Matthews RG, Ludwig ML. Nat Struct Biol. 2002 Jan;9(1):53–6. PubMed Europe PMC Scholia
- Dissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis. Castro C, Gratson AA, Evans JC, Jiracek J, Collinsová M, Ludwig ML, et al. Biochemistry. 2004 May 11;43(18):5341–51. PubMed Europe PMC Scholia
- Contribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons. Miyamoto R, Otsuguro KI, Yamaguchi S, Ito S. J Neurochem. 2014 Jul;130(1):29–40. PubMed Europe PMC Scholia