Methionine metabolism leading to Sulphur Amino Acids and related disorders (Homo sapiens)

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21GNMT deficiencyGNMTHTOxHomocysteineAMPADKDMethionineSUOXX-MTMethionine adenosyltransferase I/IIIATPDiphosphate ionSulphocysteineSarcosinePhosphate ionHypotaurineCystathionineMethyl-cobalaminCysteineGlycineCyDMSCySDBuild proteinsSulfiteS-AdenosylhomocysteineCysteine sulfinic acidMAT2AAHCYSulfateS-AdenosylmethionineBetaineBMTCBSTaurineB-Sulfinyl pyruvateCSATAdenosineMAT1AMethylation onDNA, RNA, hormones,Lipids, proteins, Creatine-P etc.SAHH deficiencyAdenosine kinase deficiencyMethyl-cobalaminMSCTHCytathionine beta-sythase deficiencyCystathionasedeficiencySulphite oxidasedeficiencyMAT deficiencyMAT2BH2O


This pathway visualises the conversion of methionine to inorganic sulphates (involving the formation of homocysteine, a toxic intermediate also related to MTHFR deficiency [1]). 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)).

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Ontology Terms



  1. Linnebank M, Lagler F, Muntau AC, Röschinger W, Olgemöller B, Fowler B, Koch HG; ''Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases.''; J Inherit Metab Dis, 2005 PubMed Europe PMC
  2. 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


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108077view11:47, 28 November 2019FehrhartOntology Term : 'disease pathway' added !
106794view13:27, 17 September 2019MaintBotHMDB identifier normalization
104020view17:36, 25 April 2019IreneHemelModified description
102744view10:56, 22 January 2019EgonwReplaced another secondary ChEBI ID with primary ID.
102743view06:45, 22 January 2019EgonwReplaced secondary ChEBI IDs with primary IDs.
102738view16:29, 21 January 2019DeSlOntology Term : 'glycine N-methyltransferase deficiency pathway' added !
102737view16:28, 21 January 2019DeSlOntology Term : 'glycine N-methyltransferase deficiency' added !
102736view16:28, 21 January 2019DeSlchanged weird symbols
102735view16:27, 21 January 2019DeSlOntology Term : 'sulfite oxidase deficiency pathway' added !
102734view16:26, 21 January 2019DeSlOntology Term : 'methionine degradation pathway' added !
102733view16:25, 21 January 2019DeSlAnnotated MS-protein
102732view16:23, 21 January 2019DeSlAnnotated more nodes, added some side metabolites
102731view15:53, 21 January 2019DeSlModified title
102730view14:49, 21 January 2019DeSlAdded last metabolite ID, and annotated PW nodes
102723view14:29, 21 January 2019DeSlConnected unconnected lines
102722view14:28, 21 January 2019DeSlAdded identifiers for metabolites
102721view14:12, 21 January 2019DeSlModified description
98565view14:18, 17 September 2018DeSlChanged layout, moved colour in node to outline of node.
98342view20:21, 23 August 2018Elisson nlUniprot References Added
98313view14:06, 20 August 2018DeSlChanged colour of nodes according to reference.
98312view14:01, 20 August 2018DeSlAdded last nodes for diseases and proteins
98311view12:59, 20 August 2018DeSlAdded literature ref, removed nodes which are not part of lit. ref, and added those that are.
97580view16:19, 26 May 2018EgonwReplaced a secondary ChEBI identifier with a primary identifier (I missed one).
97579view07:35, 26 May 2018EgonwReplaced secondary ChEBI identifiers with primary identifiers.
97457view18:23, 21 May 2018Khanspersconnected interactions
97443view07:44, 18 May 2018EgonwOntology Term : 'altered metal homeostasis pathway' added !
97442view07:43, 18 May 2018EgonwModified description
97433view15:20, 16 May 2018HRitterModified title
97432view15:20, 16 May 2018HRitterNew pathway

External references


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NameTypeDatabase referenceComment
ADKDGeneProductENSG00000156110 (Ensembl) aka Adenosine kinase
AHCYProteinP23526 (Uniprot-TrEMBL) S-adenosylhomocysteine hydrolase
AMPMetaboliteCHEBI:16027 (ChEBI)
ATPMetaboliteCHEBI:15422 (ChEBI)
AdenosineMetaboliteCHEBI:16335 (ChEBI)
B-Sulfinyl pyruvateMetaboliteHMDB0002332 (HMDB)
BMTProteinE5RJH0 (Uniprot-TrEMBL) aka Betaine--homocysteine S-methyltransferase 1
BetaineMetaboliteCHEBI:17750 (ChEBI)
Build proteinsPathwayWP3580 (WikiPathways)
CBSGeneProductENSG00000160200 (Ensembl) cystathioniune beta-synthase
CSATProteincysteine sulphinate alpha-oxoglutarate aminotransferase
CTHGeneProductENSG00000116761 (Ensembl) cystathioniune gamma-lyase
CyDProteinQ16878 (Uniprot-TrEMBL) Cysteine dioxygenase type 1
CySDProteinF8WCW3 (Uniprot-TrEMBL) Cysteine sulfinic acid decarboxylase
CystathionineMetaboliteCHEBI:17482 (ChEBI)
Cysteine sulfinic acid MetaboliteCHEBI:61085 (ChEBI)
CysteineMetaboliteCHEBI:15356 (ChEBI)
Diphosphate ionMetaboliteQ290828 (Wikidata)
GNMTProteinQ14749 (Uniprot-TrEMBL)
GlycineMetaboliteHMDB0000123 (HMDB)
H2OMetaboliteCHEBI:15377 (ChEBI)
HTOxProtein1.8.1.3 (Enzyme Nomenclature)
  • hypothaurine:NAD+ oxidoreductase
  • Two known cofactors: heme,Molybdenum
HomocysteineMetaboliteCHEBI:17230 (ChEBI)
HypotaurineMetaboliteCHEBI:16668 (ChEBI)
MAT1AProteinQ00266 (Uniprot-TrEMBL)
MAT2AProteinB4DEX8 (Uniprot-TrEMBL)
MAT2BProteinH7C0X7 (Uniprot-TrEMBL)
MSProtein5-methyltetrahydrofolate-homocysteine methyltransferase
MSProteinENSG00000116984 (Ensembl) 5-methyltetrahydrofolate-homocysteine methyltransferase
Methionine adenosyltransferase I/IIIProtein2.5.1.6 (Enzyme Nomenclature)
MethionineMetaboliteCHEBI:16643 (ChEBI)
Methyl- cobalaminMetaboliteCHEBI:28115 (ChEBI) "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"
Methylation on

DNA, RNA, hormones, Lipids, proteins,

Creatine-P etc.
PathwayWP704 (WikiPathways)
Phosphate ionMetaboliteQ177811 (Wikidata)
S-AdenosylhomocysteineMetaboliteCHEBI:16680 (ChEBI)
S-AdenosylmethionineMetaboliteCHEBI:67040 (ChEBI)
SUOXGeneProduct1.8.3.1 (Enzyme Nomenclature) Sulfite oxidase
SarcosineMetaboliteCHEBI:15611 (ChEBI)
SulfateMetaboliteHMDB0001448 (HMDB)
SulfiteMetaboliteCHEBI:26823 (ChEBI)
SulphocysteineMetaboliteCHEBI:27891 (ChEBI)
TaurineMetaboliteCHEBI:15891 (ChEBI)
X-MTProteinS-adenosylmethionine-dependent transmethylation enzymes

Annotated Interactions

No annotated interactions

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