This pathway visualises the conversion of methionine to cysteine, after which it is further metabolised in either an oxidative or non-oxidative manner. Several of these metabolites are altered in people suffering from MoCD and SO; changes in metabolite concentrations (comparing MoCD cases to controls) are highlighted in blue (see [https://www.wikipathways.org/index.php/Pathway:WP4507] for the pathway on MoCD). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).

Authors

Denise Slenter , Egon Willighagen , Irene Hemel , Daniel Domingo-Fdez , Friederike Ehrhart , and Finterly Hu

Cited In

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Organism

Homo sapiens

Communities

Inborn Errors of Metabolism (IEM) Pathways Rare Diseases

Annotations

Pathway Ontology: methionine degradation pathway cysteine and methionine metabolic pathway cysteine metabolic pathway methionine cycle/metabolic pathway disease 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. Smith AE, Matthews RG. Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase. Biochemistry. 2000 Nov 14;39(45):13880–90. PubMed Europe PMC Scholia
3. Bandarian V, Pattridge KA, Lennon BW, Huddler DP, Matthews RG, Ludwig ML. Domain alternation switches B(12)-dependent methionine synthase to the activation conformation. Nat Struct Biol. 2002 Jan;9(1):53–6. PubMed Europe PMC Scholia
4. Castro C, Gratson AA, Evans JC, Jiracek J, Collinsová M, Ludwig ML, et al. Dissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis. Biochemistry. 2004 May 11;43(18):5341–51. PubMed Europe PMC Scholia
5. Miyamoto R, Otsuguro K-I, Yamaguchi S, Ito S. Contribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons. J Neurochem. 2014 Jul;130(1):29–40. PubMed Europe PMC Scholia