Cysteine and methionine catabolism (Homo sapiens)

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14235Transsulfuration pathwayMATCystine5-Methyltetrahydrofolategamma-GlutamylcysteineTetra-hydrofolatePyrAATHypotaurineH2S-CH3H2OMSSO3 2-B-Sulfinyl pyruvateSulfateATPSQR3-Mercaptopyruvic acidS-AdenosylmethionineAATSAAHBHMTCysteineSOS-sulfocysteineGSGlutathioneMTKGCysteine sulfinic acidRSSHCO2MPSTCSDS-AdenosylhomocysteineGCSCSECystathionineHomocysteineDimethylglycineSulfiteCSESTS2O3 (2-)serineCDOMethionineGlualpha-KBTaurineO2Betaine4H2SAdenosineNH4+CSEKGGluCSEGluGlySQRSDOSOOxidative cysteine pathwayNon-Oxidative cysteine pathway


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)).

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Ontology Terms



  1. 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
  2. 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
  3. 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
  4. 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
  5. Smith AE, Matthews RG; ''Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase.''; Biochemistry, 2000 PubMed Europe PMC


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104395view14:15, 23 May 2019IreneHemelAdded more Rhea IDs
104394view13:59, 23 May 2019IreneHemelAdded Rhea IDs
104241view15:16, 11 May 2019MaintBotRemoved the RHEA: prefix from the identifier.
104174view16:12, 8 May 2019DeSlAdded Rhea IDs for reactions from homocysteine to methionine
104173view15:50, 8 May 2019DeSlMade blue arrows smaller, for better visualisation on website.
104017view15:29, 25 April 2019IreneHemelModified description
104013view15:28, 25 April 2019IreneHemelAdded more Rhea IDs
104012view15:18, 25 April 2019IreneHemelAdded more Rhea IDs
104009view14:28, 25 April 2019IreneHemelAdded Rhea IDs and correct CHEBI or Uniprot IDs for several reactions
102835view20:44, 24 January 2019EgonwReplaced secondary ChEBI IDs with primary IDs.
102826view15:47, 24 January 2019DeSlModified description
102812view15:19, 24 January 2019DeSlAnother small layout change
102811view15:18, 24 January 2019DeSlChanged layout
102810view15:17, 24 January 2019DeSlChanged Enzyme nomenclature ID to Uniprot for SO
102799view13:57, 24 January 2019DeSlOntology Term : 'methionine degradation pathway' added !
102798view13:56, 24 January 2019DeSlOntology Term : 'methionine cycle/metabolic pathway' added !
102797view13:56, 24 January 2019DeSlOntology Term : 'cysteine metabolic pathway' added !
102796view13:56, 24 January 2019DeSlOntology Term : 'cysteine and methionine metabolic pathway' added !
102795view13:54, 24 January 2019DeSlModified description
102794view13:52, 24 January 2019DeSlAdded in- and decreased metabolites related to MoCD.
102793view13:48, 24 January 2019DeSlAnnotated RSSH
102791view13:44, 24 January 2019DeSlChanged layout according to lit. ref
102790view13:40, 24 January 2019DeSlAnnotated several proteins (bottom)
102789view13:34, 24 January 2019DeSlLayout update
102788view13:33, 24 January 2019DeSlUpdated other missing reactions
102786view12:02, 24 January 2019DeSlAdded more side metabolites
102785view10:53, 24 January 2019DeSlmore layout changes, added side metabolites to left bottom side of PW.
102784view10:31, 24 January 2019DeSlChanged layout top, annotated side metabolites top
102749view07:16, 23 January 2019EgonwReplaced secondary ChEBI IDs with primary IDs.
102747view13:35, 22 January 2019DeSlAdded several side metabolites, and lit. ref.
102741view16:36, 21 January 2019DeSlModified description
102740view16:34, 21 January 2019DeSlNew pathway

External references


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NameTypeDatabase referenceComment
-CH3MetaboliteCHEBI:32875 (ChEBI)
3-Mercaptopyruvic acidMetaboliteCHEBI:57678 (ChEBI)
5-Methyl tetrahydrofolateMetaboliteCHEBI:15641 (ChEBI)
AATProteinA0A140VK69 (Uniprot-TrEMBL) AKA Aspartate aminotransferase
AATProteinP17174 (Uniprot-TrEMBL) AKA Aspartate aminotransferase
ATPMetaboliteCHEBI:30616 (ChEBI)
AdenosineMetaboliteCHEBI:16335 (ChEBI)
B-Sulfinyl pyruvateMetaboliteHMDB02332 (HMDB)
BHMTProteinQ93088 (Uniprot-TrEMBL) AKA Betaine--homocysteine S-methyltransferase 1
BetaineMetaboliteCHEBI:17750 (ChEBI)
CDOProteinQ16878 (Uniprot-TrEMBL) Cysteine dioxygenase type 1
CO2MetaboliteCHEBI:16526 (ChEBI)
CSDProteinF8WCW3 (Uniprot-TrEMBL) AKA Cysteine sulfinic acid decarboxylase
CSEProteinP32929 (Uniprot-TrEMBL) Cystathionine gamma-lyase
CSEProteinP35520 (Uniprot-TrEMBL) aka Cystathionine beta-synthase
CystathionineMetaboliteCHEBI:58161 (ChEBI)
Cysteine sulfinic acid MetaboliteCHEBI:61085 (ChEBI)
CysteineMetaboliteCHEBI:35235 (ChEBI)
CystineMetaboliteCHEBI:17376 (ChEBI)
DimethylglycineMetaboliteHMDB00092 (HMDB)
GCSProteinQ14TF0 (Uniprot-TrEMBL) AKA Glutamate-cysteine ligase
GSProteinP48637 (Uniprot-TrEMBL) AKA Glutathione synthetase
GluMetaboliteCHEBI:28300 (ChEBI) AKA glutamine
GluMetaboliteCHEBI:29985 (ChEBI) AKA glutamine
GlutathioneMetaboliteCHEBI:57925 (ChEBI)
GlyMetaboliteCHEBI:57305 (ChEBI) AKA glycine
H2OMetaboliteCHEBI:15377 (ChEBI)
H2SMetaboliteCHEBI:16136 (ChEBI) aka Hydrogen sulfide
H2SMetaboliteCHEBI:29919 (ChEBI) aka Hydrogen sulfide
HomocysteineMetaboliteCHEBI:58199 (ChEBI)
HypotaurineMetaboliteCHEBI:16668 (ChEBI)
KGMetaboliteCHEBI:16810 (ChEBI) aka a-ketoglutarate (a-KG)
MATGeneProductQ00266 (Uniprot-TrEMBL) aka Methionine adenosyltransferase
MPSTProteinP25325 (Uniprot-TrEMBL) AKA 3-mercaptopyruvate sulfurtransferase
MSProteinQ99707 (Uniprot-TrEMBL) AKA Methionine synthase
MTGeneProduct2.1.1 (BRENDA) AKA Methyltransferase
MethionineMetaboliteCHEBI:57844 (ChEBI)
NH4+MetaboliteCHEBI:28938 (ChEBI)
O2MetaboliteCHEBI:15379 (ChEBI)
PyrMetaboliteCHEBI:15361 (ChEBI) AKA Pyruvic acid
RSSHMetaboliteCHEBI:30605 (ChEBI) AKA persulfurated thiol-containing compound (RSSH)
S-AdenosylhomocysteineMetaboliteCHEBI:57856 (ChEBI)
S-AdenosylmethionineMetaboliteCHEBI:59789 (ChEBI)
S-sulfocysteineMetaboliteCHEBI:27891 (ChEBI)
S2O3 (2-)MetaboliteQ410739 (Wikidata) AKA thiosulfate ion
SAAHProteinP23526 (Uniprot-TrEMBL) AKA Adenosylhomocysteinase
SDOProteinO95571 (Uniprot-TrEMBL) AKA sulfur dioxygenase
SOGeneProduct1.8.3.1 (BRENDA) AKA Sulfite oxidase
SO3 2-MetaboliteQ413363 (Wikidata) AKA sulfite ion.
SOGeneProductP51687 (Uniprot-TrEMBL) AKA Sulfite oxidase
SQRProteinQ9Y6N5 (Uniprot-TrEMBL)
STProteinAKA sulfur transferase
SulfateMetaboliteHMDB01448 (HMDB)
SulfiteMetaboliteCHEBI:26823 (ChEBI)
TaurineMetaboliteCHEBI:15891 (ChEBI)
Tetra- hydrofolateMetaboliteCHEBI:20506 (ChEBI)
Transsulfuration pathwayPathway
alpha-KBMetaboliteCHEBI:16763 (ChEBI)
gamma-GlutamylcysteineMetaboliteCHEBI:58173 (ChEBI)
serineMetaboliteCHEBI:33384 (ChEBI)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
3-Mercaptopyruvic acidH2Smim-conversion21741 (Rhea)
ATPmim-conversion21081 (Rhea)
Adenosinemim-conversion21709 (Rhea)
CystathionineCysteinemim-conversion14006 (Rhea)
Cysteine3-Mercaptopyruvic acidmim-conversion17442 (Rhea)
CysteineCysteine sulfinic acid mim-conversion20442 (Rhea)
CysteineCystinemim-conversion20598 (Rhea)
CysteineH2Smim-conversion20916 (Rhea)
Cysteinegamma-Glutamylcysteinemim-conversion13286 (Rhea)
CystineCysteinemim-conversion20599 (Rhea)
Glumim-conversion13286 (Rhea)
Glumim-conversion17442 (Rhea)
Glymim-conversion13558 (Rhea)
HomocysteineCystathioninemim-conversion10113 (Rhea)
Homocysteinemim-conversionRHEA:11173 (Rhea)
Homocysteinemim-conversionRHEA:22337 (Rhea)
KGmim-conversion17442 (Rhea)
MethionineS-Adenosylmethioninemim-conversion21081 (Rhea)
O2mim-conversion20442 (Rhea)
Pyrmim-conversion21741 (Rhea)
S-AdenosylhomocysteineHomocysteinemim-conversion21709 (Rhea)
gamma-GlutamylcysteineGlutathionemim-conversion13558 (Rhea)
mim-conversion14006 (Rhea)
serinemim-conversion10113 (Rhea)
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