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)).befAKA Glutathione synthetaseaka a-ketoglutarate (a-KG)AKA Methionine synthaseAKA 3-mercaptopyruvate sulfurtransferaseAKA Glutamate-cysteine ligaseaka Methionine adenosyltransferaseAKA AdenosylhomocysteinaseAKA sulfite ion.Cysteine dioxygenase type 1AKA Cysteine sulfinic acid decarboxylaseAKA Sulfite oxidaseaf8aka Cystathionine beta-synthaseAKA Aspartate aminotransferaseaka Hydrogen sulfideAKA MethyltransferaseAKA Betaine--homocysteine S-methyltransferase 1Cystathionine gamma-lyaseAKA Pyruvic acidAKA sulfur transferaseAKA quinone oxidoreductaseAKA glutamineAKA thiosulfate ionAKA Aspartate aminotransferaseAKA persulfurated thiol-containing compound (RSSH)aka Hydrogen sulfideaka Cystathionine beta-synthaseaka a-ketoglutarate (a-KG)AKA glutamineCystathionine gamma-lyaseAKA glutamineAKA glycineAKA sulfur dioxygenaseAKA Sulfite oxidaseb81dd5b81eb2af8methionine degradation pathwayPW:0001302Pathway Ontologycysteine and methionine metabolic pathwayPW:0001078Pathway Ontologycysteine metabolic pathwayPW:0000049Pathway Ontologymethionine cycle/metabolic pathwayPW:0000048Pathway Ontologydisease pathwayPW:0000013Pathway Ontology9783642403361ISBN'Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic DiseasesSpringer-Verlag Berlin Heidelberg2014Nenad BlauMarinus DuranK Michael GibsonCarlo Dionisi-Vici11076529PubMedProtonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase.Biochemistry2000Smith AEMatthews RG15122900PubMedDissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis.Biochemistry2004Castro CGratson AAEvans JCJiracek JCollinsová MLudwig MLGarrow TA24611772PubMedContribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons.J Neurochem2014Miyamoto ROtsuguro KYamaguchi SIto S11731805PubMedDomain alternation switches B(12)-dependent methionine synthase to the activation conformation.Nat Struct Biol2002Bandarian VPattridge KALennon BWHuddler DPMatthews RGLudwig ML