Methionine metabolism leading to sulfur amino acids and related disorders (Homo sapiens)

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21GNMT deficiencyHomocysteineS-AdenosylhomocysteineSarcosineTaurineAdenosineCySDCyDAMPB-Sulfinyl pyruvateADKDGNMTGlycineMethionineSulfateHypotaurineMethionine adenosyltransferase I/IIIBetaineSUOXBuild proteinsHTOxBMTCystathionineCysteineMSCBSCysteine sulfinic acidATPSulfiteCSATSulphocysteineAHCYMAT2AMAT1AS-AdenosylmethioninePhosphate ionX-MTMethyl-cobalaminDiphosphate ionMethylation onDNA, RNA, hormones,Lipids, proteins, Creatine-P etc.SAHH deficiencyAdenosine kinase deficiencyMethyl-cobalaminMSCTHCytathionine beta-sythase deficiencyCystathionasedeficiencySulphite oxidasedeficiencyMAT deficiencyMAT2BH2O


Description

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 978-3-642-40337-8).

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Quality Tags

Ontology Terms

 

Bibliography

  1. 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
  2. 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 Scholia
  3. Griffith OW; ''Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate.''; J Biol Chem, 1983 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
139560view07:06, 21 June 2025EgonwModified description
139558view07:05, 21 June 2025EgonwWater with unicode
137937view18:34, 10 March 2025KhanspersModified description
128219view04:02, 29 January 2024EweitzSoften disease color, standardize case
124957view08:37, 7 January 2023EgonwFixed the OMIM link patterns
124270view12:35, 6 October 2022DeSlConverted all interactions to textLabels to graphical lines
121757view12:03, 3 March 2022DeSlAdded edition nr to book ref.
120406view09:20, 30 November 2021Fehrhartboxed pathway nodes
119257view19:46, 22 June 2021FinterlyAdded ISBN for book citation
119254view19:39, 22 June 2021FinterlyAdded ISBN for book citation
116639view11:28, 9 May 2021EweitzModified title
114510view19:56, 18 January 2021DeSlAdded EC number for CSAT
114509view19:50, 18 January 2021DeSlUpdated complex to graphical visualisation.
114496view13:27, 17 January 2021DeSlAdded lit. ref for CSAT conversion.
114495view13:20, 17 January 2021DeSlAdded rhea ID for homocysteine -> methionine interaction.
114494view13:18, 17 January 2021DeSlUpdated ID for MS to Uniprot.
114493view13:15, 17 January 2021DeSlAdded rhea for CySD interaction, updated Uniprot ID to reviewed.
114427view15:38, 10 January 2021DeSlUpdated complex vis. in legend.
114426view15:36, 10 January 2021DeSlUpdated visual style of PW for webview.
114425view15:34, 10 January 2021DeSlAdded two more alternative names for disease nodes.
114424view15:33, 10 January 2021DeSlOntology Term : 'hypermethioninemia due to adenosine kinase deficiency' added !
114423view15:32, 10 January 2021DeSlOntology Term : 'isolated sulfite oxidase deficiency' added !
114422view15:31, 10 January 2021DeSlalt. name for Cystathionine beta-synthase deficiency.
114421view15:30, 10 January 2021DeSlOntology Term : 'cystathioninuria pathway' added !
114420view15:30, 10 January 2021DeSlOntology Term : 'cystathioninuria' added !
114419view15:28, 10 January 2021DeSlOntology Term : 'homocystinuria pathway' added !
114418view15:28, 10 January 2021DeSlOntology Term : 'homocystinuria' added !
114417view15:26, 10 January 2021DeSlAdded alt. name for MAT deficiency.
114416view15:24, 10 January 2021DeSlOntology Term : 'cysteine and methionine metabolic pathway' added !
114412view15:17, 10 January 2021DeSlAdded some alternative names for diseases as comments; checked all OMIM IDs.
114411view16:38, 9 January 2021DeSlAdded info on SAHH deficiency.
114410view16:37, 9 January 2021DeSlOntology Term : 'hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase' added !
114409view16:36, 9 January 2021DeSlOntology Term : 'hypermethioninemia' added !
114408view16:35, 9 January 2021DeSlOntology Term : 'hypermethioninemia pathway' added !
114407view16:35, 9 January 2021DeSlOntology Term : 'hypermethioninemia pathway' added !
114406view16:34, 9 January 2021DeSlAdded links to Vit. B12 PW from IEM-portal chapter 13 Blau book
114405view16:29, 9 January 2021DeSlAdded legend
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

External references

DataNodes

View all...
NameTypeDatabase referenceComment
ADKDGeneProductENSG00000156110 (Ensembl) aka Adenosine kinase
AHCYProteinP23526 (Uniprot-TrEMBL) S-adenosylhomocysteine hydrolase
AMPMetaboliteCHEBI:16027 (ChEBI)
ATPMetaboliteCHEBI:15422 (ChEBI)
AdenosineMetaboliteCHEBI:16335 (ChEBI)
B-Sulfinyl pyruvateMetaboliteHMDB02332 (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)
GlycineMetaboliteHMDB00123 (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" https://en.wikipedia.org/wiki/Methylcobalamin
Methyl- cobalaminMetaboliteCHEBI:6869 (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" https://en.wikipedia.org/wiki/Methylcobalamin
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)
SulfateMetaboliteHMDB01448 (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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