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

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CysteineHomocysteineSulfiteMethyltransferaseAspartate aminotransferase5-MethyltetrahydrofolateGlutathione synthetaseSulfite oxidaseS-AdenosylhomocysteineCysteine sulfinic acid decarboxylaseCysteine sulfinic acidTaurineGlutamate-cysteine ligasegamma-GlutamylcysteineCystathionine beta-synthaseCystineMethionine3-mercaptopyruvate sulfurtransferaseSulfateMethionine adenosyltransferaseGlutathioneHydrogen sulfideMethionine synthase3-Mercaptopyruvic acidCystathionineTetrahydrofolic acidAdenosylhomocysteinaseS-AdenosylmethionineAspartate aminotransferaseB-Sulfinyl pyruvateCystathionine gamma-lyaseBetaineCysteine dioxygenase type 1DimethylglycineS-sulfocysteineBetaine--homocysteine S-methyltransferase 1HypotaurineHydrogen sulfide


Description

Cysteine pathway altered by molybdenum cofactor disorders

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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
3-Mercaptopyruvic acidMetaboliteCHEBI:16208 (ChEBI)
3-mercaptopyruvate sulfurtransferaseProteinP25325 (Uniprot-TrEMBL)
5-MethyltetrahydrofolateMetaboliteCHEBI:2097 (ChEBI)
AdenosylhomocysteinaseProteinA0A024R0A8 (Uniprot-TrEMBL)
Aspartate aminotransferaseProteinA0A140VK69 (Uniprot-TrEMBL)
B-Sulfinyl pyruvateMetaboliteHMDB02332 (HMDB)
Betaine--homocysteine S-methyltransferase 1ProteinE5RJH0 (Uniprot-TrEMBL)
BetaineMetaboliteCHEBI:3073 (ChEBI)
Cystathionine beta-synthaseProteinP35520 (Uniprot-TrEMBL)
Cystathionine gamma-lyaseProteinP32929 (Uniprot-TrEMBL)
CystathionineMetaboliteCHEBI:17482 (ChEBI)
Cysteine dioxygenase type 1ProteinQ16878 (Uniprot-TrEMBL)
Cysteine sulfinic acid MetaboliteCHEBI:61085 (ChEBI)
Cysteine sulfinic acid decarboxylaseProteinF8WCW3 (Uniprot-TrEMBL)
CysteineMetaboliteCHEBI:4050 (ChEBI)
CystineMetaboliteCHEBI:4052 (ChEBI)
DimethylglycineMetaboliteHMDB00092 (HMDB)
Glutamate-cysteine ligaseProteinQ14TF0 (Uniprot-TrEMBL)
Glutathione synthetaseProteinP48637 (Uniprot-TrEMBL)
GlutathioneMetaboliteCHEBI:5437 (ChEBI)
HomocysteineMetaboliteCHEBI:5751 (ChEBI)
Hydrogen sulfideMetaboliteCHEBI:5787 (ChEBI)
HypotaurineMetaboliteCHEBI:5839 (ChEBI)
Methionine adenosyltransferaseGeneProduct2.5.1.6 (BRENDA)
Methionine synthaseProteinQ99707 (Uniprot-TrEMBL)
MethionineMetaboliteCHEBI:16643 (ChEBI)
MethyltransferaseGeneProduct2.1.1 (BRENDA)
S-AdenosylhomocysteineMetaboliteCHEBI:8945 (ChEBI)
S-AdenosylmethionineMetaboliteCHEBI:67040 (ChEBI)
S-sulfocysteineMetaboliteCHEBI:27891 (ChEBI)
SulfateMetaboliteHMDB01448 (HMDB)
Sulfite oxidaseGeneProduct1.8.3.1 (BRENDA)
SulfiteMetaboliteCHEBI:26823 (ChEBI)
TaurineMetaboliteCHEBI:9406 (ChEBI)
Tetrahydrofolic acidMetaboliteCHEBI:20506 (ChEBI)
gamma-GlutamylcysteineMetaboliteCHEBI:10570 (ChEBI)

Annotated Interactions

No annotated interactions

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