This pathway visualises the conversion of methionine to inorganic sulphates (involving the formation of homocysteine, a toxic intermediate also related to MTHFR deficiency [https://www.wikipathways.org/index.php/Pathway: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

Cited In

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Organism

Homo sapiens

Communities

Inborn Errors of Metabolism (IEM) Pathways Rare Diseases

Annotations

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

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

Participants

References

1. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Springer; 2014. 867 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
2. Griffith OW. Cysteinesulfinate metabolism. altered partitioning between transamination and decarboxylation following administration of beta-methyleneaspartate. J Biol Chem. 1983 Feb 10;258(3):1591–8. PubMed Europe PMC Scholia
3. Linnebank M, Lagler F, Muntau AC, Röschinger W, Olgemöller B, Fowler B, et al. Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. J Inherit Metab Dis. 2005;28(6):1167–8. PubMed Europe PMC Scholia