Cysteine and methionine catabolism (Homo sapiens)
From WikiPathways
Description
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 [1] for the pathway on MoCD).
This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).Quality Tags
Ontology Terms
Bibliography
- Nenad Blau, Marinus Duran, K Michael Gibson, Carlo Dionisi-Vici; '''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; Springer-Verlag Berlin Heidelberg, 2014
- Miyamoto R, Otsuguro K, Yamaguchi S, Ito S; ''Contribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons.''; J Neurochem, 2014 PubMed Europe PMC Scholia
- Smith AE, Matthews RG; ''Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase.''; Biochemistry, 2000 PubMed Europe PMC Scholia
- Bandarian V, Pattridge KA, Lennon BW, Huddler DP, Matthews RG, Ludwig ML; ''Domain alternation switches B(12)-dependent methionine synthase to the activation conformation.''; Nat Struct Biol, 2002 PubMed Europe PMC Scholia
- Castro C, Gratson AA, Evans JC, Jiracek J, Collinsová M, Ludwig ML, Garrow TA; ''Dissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis.''; Biochemistry, 2004 PubMed Europe PMC Scholia
History
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External references
DataNodes
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Name | Type | Database reference | Comment |
---|---|---|---|
-CH3 | Metabolite | CHEBI:32875 (ChEBI) | |
3-Mercaptopyruvic acid | Metabolite | CHEBI:57678 (ChEBI) | |
5-Methyl tetrahydrofolate | Metabolite | CHEBI:15641 (ChEBI) | |
AAT | Protein | A0A140VK69 (Uniprot-TrEMBL) | AKA Aspartate aminotransferase |
AAT | Protein | P17174 (Uniprot-TrEMBL) | AKA Aspartate aminotransferase |
ATP | Metabolite | CHEBI:30616 (ChEBI) | |
Adenosine | Metabolite | CHEBI:16335 (ChEBI) | |
B-Sulfinyl pyruvate | Metabolite | HMDB02332 (HMDB) | |
BHMT | Protein | E5RJH0 (Uniprot-TrEMBL) | AKA Betaine--homocysteine S-methyltransferase 1 |
Betaine | Metabolite | CHEBI:17750 (ChEBI) | |
CDO | Protein | Q16878 (Uniprot-TrEMBL) | Cysteine dioxygenase type 1 |
CO2 | Metabolite | CHEBI:16526 (ChEBI) | |
CSD | Protein | F8WCW3 (Uniprot-TrEMBL) | AKA Cysteine sulfinic acid decarboxylase |
CSE | Protein | P32929 (Uniprot-TrEMBL) | Cystathionine gamma-lyase |
CSE | Protein | P35520 (Uniprot-TrEMBL) | aka Cystathionine beta-synthase |
Cystathionine | Metabolite | CHEBI:58161 (ChEBI) | |
Cysteine sulfinic acid | Metabolite | CHEBI:61085 (ChEBI) | |
Cysteine | Metabolite | CHEBI:35235 (ChEBI) | |
Cystine | Metabolite | CHEBI:17376 (ChEBI) | |
Dimethylglycine | Metabolite | HMDB00092 (HMDB) | |
GCS | Protein | Q14TF0 (Uniprot-TrEMBL) | AKA Glutamate-cysteine ligase |
GS | Protein | P48637 (Uniprot-TrEMBL) | AKA Glutathione synthetase |
Glu | Metabolite | CHEBI:28300 (ChEBI) | AKA glutamine |
Glu | Metabolite | CHEBI:29985 (ChEBI) | AKA glutamine |
Glutathione | Metabolite | CHEBI:57925 (ChEBI) | |
Gly | Metabolite | CHEBI:57305 (ChEBI) | AKA glycine |
H2O | Metabolite | CHEBI:15377 (ChEBI) | |
H2S | Metabolite | CHEBI:16136 (ChEBI) | aka Hydrogen sulfide |
H2S | Metabolite | CHEBI:29919 (ChEBI) | aka Hydrogen sulfide |
Homocysteine | Metabolite | CHEBI:58199 (ChEBI) | |
Hypotaurine | Metabolite | CHEBI:16668 (ChEBI) | |
KG | Metabolite | CHEBI:16810 (ChEBI) | aka a-ketoglutarate (a-KG) |
MAT | GeneProduct | Q00266 (Uniprot-TrEMBL) | aka Methionine adenosyltransferase |
MPST | Protein | P25325 (Uniprot-TrEMBL) | AKA 3-mercaptopyruvate sulfurtransferase |
MS | Protein | Q99707 (Uniprot-TrEMBL) | AKA Methionine synthase |
MT | GeneProduct | 2.1.1 (BRENDA) | AKA Methyltransferase |
Methionine | Metabolite | CHEBI:57844 (ChEBI) | |
NH4+ | Metabolite | CHEBI:28938 (ChEBI) | |
O2 | Metabolite | CHEBI:15379 (ChEBI) | |
Pyr | Metabolite | CHEBI:15361 (ChEBI) | AKA Pyruvic acid |
RSSH | Metabolite | CHEBI:30605 (ChEBI) | AKA persulfurated thiol-containing compound (RSSH) |
S-Adenosylhomocysteine | Metabolite | CHEBI:57856 (ChEBI) | |
S-Adenosylmethionine | Metabolite | CHEBI:59789 (ChEBI) | |
S-sulfocysteine | Metabolite | CHEBI:27891 (ChEBI) | |
S2O3 (2-) | Metabolite | Q410739 (Wikidata) | AKA thiosulfate ion |
SAAH | Protein | P23526 (Uniprot-TrEMBL) | AKA Adenosylhomocysteinase |
SDO | Protein | O95571 (Uniprot-TrEMBL) | AKA sulfur dioxygenase |
SO | GeneProduct | 1.8.3.1 (BRENDA) | AKA Sulfite oxidase |
SO3 2- | Metabolite | Q413363 (Wikidata) | AKA sulfite ion. |
SO | GeneProduct | P51687 (Uniprot-TrEMBL) | AKA Sulfite oxidase |
SQR | Protein | Q9Y6N5 (Uniprot-TrEMBL) | |
ST | Protein | AKA sulfur transferase | |
Sulfate | Metabolite | HMDB01448 (HMDB) | |
Sulfite | Metabolite | CHEBI:26823 (ChEBI) | |
Taurine | Metabolite | CHEBI:15891 (ChEBI) | |
Tetra- hydrofolate | Metabolite | CHEBI:20506 (ChEBI) | |
alpha-KB | Metabolite | CHEBI:16763 (ChEBI) | |
gamma-Glutamylcysteine | Metabolite | CHEBI:58173 (ChEBI) | |
serine | Metabolite | CHEBI:33384 (ChEBI) |
Annotated Interactions
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Source | Target | Type | Database reference | Comment |
---|---|---|---|---|
3-Mercaptopyruvic acid | H2S | mim-conversion | 21741 (Rhea) | |
ATP | mim-conversion | 21081 (Rhea) | ||
Adenosine | mim-conversion | 21709 (Rhea) | ||
Cystathionine | Cysteine | mim-conversion | 14006 (Rhea) | |
Cysteine | 3-Mercaptopyruvic acid | mim-conversion | 17442 (Rhea) | |
Cysteine | Cysteine sulfinic acid | mim-conversion | 20442 (Rhea) | |
Cysteine | Cystine | mim-conversion | 20598 (Rhea) | |
Cysteine | H2S | mim-conversion | 20916 (Rhea) | |
Cysteine | gamma-Glutamylcysteine | mim-conversion | 13286 (Rhea) | |
Cystine | Cysteine | mim-conversion | 20599 (Rhea) | |
Glu | mim-conversion | 13286 (Rhea) | ||
Glu | mim-conversion | 17442 (Rhea) | ||
Gly | mim-conversion | 13558 (Rhea) | ||
Homocysteine | Cystathionine | mim-conversion | 10113 (Rhea) | |
KG | mim-conversion | 17442 (Rhea) | ||
Methionine | S-Adenosylmethionine | mim-conversion | 21081 (Rhea) | |
O2 | mim-conversion | 20442 (Rhea) | ||
Pyr | mim-conversion | 21741 (Rhea) | ||
S-Adenosylhomocysteine | Homocysteine | mim-conversion | 21709 (Rhea) | |
gamma-Glutamylcysteine | Glutathione | mim-conversion | 13558 (Rhea) | |
mim-conversion | 14006 (Rhea) | |||
serine | mim-conversion | 10113 (Rhea) |