Methionine metabolism leading to sulfur amino acids and related disorders (WP4292)

Homo sapiens

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This pathway visualises the conversion of methionine to inorganic sulphates (involving the formation of homocysteine, a toxic intermediate also related to MTHFR deficiency WP4288). 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)).

Authors

Hermann Ritter , Egon Willighagen , Kristina Hanspers , Denise Slenter , Elisson nl , Irene Hemel , Friederike Ehrhart , Eric Weitz , and Finterly Hu

Activity

last edited

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Cited In

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Organisms

Homo sapiens

Communities

Inherited Metabolic Disorders (IMD) Pathways Rare Diseases

Annotations

Disease Ontology

hypermethioninemia due to adenosine kinase deficiency hypermethioninemia cystathioninuria hypermethioninemia with deficiency of S-adenosylhomocysteine hydrolase homocystinuria isolated sulfite oxidase deficiency glycine N-methyltransferase deficiency

Pathway Ontology

homocystinuria pathway glycine N-methyltransferase deficiency pathway cysteine and methionine metabolic pathway disease pathway cystathioninuria pathway methionine degradation pathway hypermethioninemia pathway hypermethioninemia pathway sulfite oxidase deficiency pathway altered metal homeostasis pathway

Participants
Query Drugst.One

Label Type Compact URI Comment
Methionine Metabolite chebi:16643
Sulphocysteine Metabolite chebi:27891
Cystathionine Metabolite chebi:17482
Sulfate Metabolite hmdb:HMDB0001448
Taurine Metabolite chebi:15891
Sulfite Metabolite chebi:26823
S-Adenosylmethionine Metabolite chebi:67040
Cysteine Metabolite chebi:15356
Adenosine Metabolite chebi:16335
S-Adenosylhomocysteine Metabolite chebi:16680
Methyl-cobalamin Metabolite chebi:28115 '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
Hypotaurine Metabolite chebi:16668
Glycine Metabolite hmdb:HMDB0000123
Betaine Metabolite chebi:17750
Cysteine sulfinic acid Metabolite chebi:61085 aka 3-sulfino-L-alanine
ATP Metabolite chebi:15422
AMP Metabolite chebi:16027
Sarcosine Metabolite chebi:15611
Diphosphate ion Metabolite wikidata:Q290828
Beta-sulfinyl pyruvate Metabolite hmdb:HMDB0002332
Homocysteine Metabolite chebi:17230
Phosphate ion Metabolite wikidata:Q177811
H2O Metabolite chebi:15377
ADKD GeneProduct ensembl:ENSG00000156110 aka Adenosine kinase
SUOX GeneProduct eccode:1.8.3.1 Sulfite oxidase
CBS GeneProduct ensembl:ENSG00000160200 cystathioniune beta-synthase
CTH GeneProduct ensembl:ENSG00000116761 cystathioniune gamma-lyase
CSAT Protein eccode:2.6.1.- cysteine sulphinate alpha-oxoglutarate aminotransferase
Not available in Uniprot (yet) 2021-01-17
Methionine adenosyltransferase I/III Protein eccode:2.5.1.6
CyD Protein uniprot:Q16878 Cysteine dioxygenase type 1
CySD Protein uniprot:Q9Y600 Cysteine sulfinic acid decarboxylase
HTOx Protein eccode:1.8.1.3 hypothaurine:NAD+ oxidoreductase
Two known cofactors: heme,Molybdenum
MS Protein uniprot:Q99707 5-methyltetrahydrofolate-homocysteine methyltransferase
BMT Protein uniprot:E5RJH0 aka Betaine--homocysteine S-methyltransferase 1
MAT2A Protein uniprot:B4DEX8
AHCY Protein uniprot:P23526 S-adenosylhomocysteine hydrolase
MAT1A Protein uniprot:Q00266
GNMT Protein uniprot:Q14749
MAT2B Protein uniprot:H7C0X7

References

  1. Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Springer; 2014. 0 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
  2. Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate. Griffith OW. J Biol Chem. 1983 Feb 10;258(3):1591–8. PubMed Europe PMC Scholia
  3. Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. Linnebank M, Lagler F, Muntau AC, Röschinger W, Olgemöller B, Fowler B, et al. J Inherit Metab Dis. 2005;28(6):1167–8. PubMed Europe PMC Scholia