Cysteine and methionine catabolism (WP4504)

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 [] for the pathway on MoCD). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).
last edited


DeSl, MaintBot, IreneHemel, Ddomingof, Fehrhart, and Finterly

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Pathway Ontology: methionine degradation pathway cysteine and methionine metabolic pathway cysteine metabolic pathway methionine cycle/metabolic pathway disease pathway


Label Type Compact Identifier
Transsulfuration pathway Pathway None
Methionine Metabolite chebi:57844
Tetra-hydrofolate Metabolite chebi:57453
Cystathionine Metabolite chebi:58161
-CH3 Metabolite chebi:32875
ATP Metabolite chebi:30616
GS Protein uniprot:P48637
Sulfate Metabolite chebi:16189
KG Metabolite chebi:16810
Taurine Metabolite chebi:15891
3-Mercaptopyruvic acid Metabolite chebi:57678
Sulfite Metabolite chebi:17359
MS Protein uniprot:Q99707
S-Adenosylmethionine Metabolite chebi:59789
MPST Protein uniprot:P25325
GCS Protein uniprot:Q14TF0
Cysteine Metabolite chebi:35235
CO2 Metabolite chebi:16526
MAT GeneProduct uniprot:Q00266
SAAH Protein uniprot:P23526
SO3 2- Metabolite chebi:17359
CDO Protein uniprot:Q16878
Glutathione Metabolite chebi:57925
CSD Protein uniprot:Q9Y600
SO GeneProduct uniprot:P51687
S-Adenosylhomocysteine Metabolite chebi:57856
Dimethylglycine Metabolite chebi:58251
5-Methyltetrahydrofolate Metabolite chebi:18608
CSE Protein uniprot:P35520
Hypotaurine Metabolite chebi:57853
AAT Protein uniprot:A0A140VK69
gamma-Glutamylcysteine Metabolite chebi:58173
Cystine Metabolite chebi:17376
H2S Metabolite chebi:29919
MT GeneProduct brenda:2.1.1
BHMT Protein uniprot:Q93088
H2O Metabolite chebi:15377
CSE Protein uniprot:P32929
S-sulfocysteine Metabolite chebi:27891
Betaine Metabolite chebi:17750
alpha-KB Metabolite chebi:16763
Pyr Metabolite chebi:15361
Cysteine sulfinic acid Metabolite chebi:61085
ST Protein None
SQR Protein uniprot:Q9Y6N5
serine Metabolite chebi:33384
Glu Metabolite chebi:29985
O2 Metabolite chebi:15379
thiosulfate(2−) Metabolite chebi:16094
AAT Protein uniprot:P17174
B-Sulfinyl pyruvate Metabolite hmdb:HMDB0002332
Homocysteine Metabolite chebi:58199
RSSH Metabolite chebi:30605
H2S Metabolite chebi:16136
Adenosine Metabolite chebi:16335
NH4+ Metabolite chebi:28938
CSE Protein uniprot:P35520
KG Metabolite chebi:16810
Glu Metabolite chebi:28300
CSE Protein uniprot:P32929
Glu Metabolite chebi:29985
Gly Metabolite chebi:57305
SQR Protein uniprot:Q9Y6N5
SDO Protein uniprot:O95571
SO GeneProduct brenda:


  1. Smith AE, Matthews RG. Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase. Biochemistry. 2000 Nov 14;39(45):13880–90. PubMed Europe PMC Scholia
  2. 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 Jan;9(1):53–6. PubMed Europe PMC Scholia
  3. Castro C, Gratson AA, Evans JC, Jiracek J, Collinsová M, Ludwig ML, et al. Dissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis. Biochemistry. 2004 May 11;43(18):5341–51. PubMed Europe PMC Scholia
  4. Miyamoto R, Otsuguro K-I, Yamaguchi S, Ito S. Contribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons. J Neurochem. 2014 Jul;130(1):29–40. PubMed Europe PMC Scholia
  5. PubMed Europe PMC Scholia