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Proteins/UNIPROT:P34897
Catalyzes the cleavage of serine to glycine accompanied with the production of 5,10-methylenetetrahydrofolate, an essential intermediate for purine biosynthesis. Serine provides the major source of folate one-carbon in cells by catalyzing the transfer of one carbon from serine to tetrahydrofolate. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway via its role in glycine and tetrahydrofolate metabolism: thymidylate biosynthesis is required to prevent uracil accumulation in mtDNA. Also required for mitochondrial translation by producing 5,10-methylenetetrahydrofolate; 5,10-methylenetetrahydrofolate providing methyl donors to produce the taurinomethyluridine base at the wobble position of some mitochondrial tRNAs. Associates with mitochondrial DNA. In addition to its role in mitochondria, also plays a role in the deubiquitination of target proteins as component of the BRISC complex: required for IFNAR1 deubiquitination by the BRISC complex..
Function from UniProtKB (P34897), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 1 · substrate of
1 compound
1 compound.
0 reported routes1 assembled route
2 rows
151 statements
151 statements · 9 reference databases · Subject: SHMT2 · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
4 reactions
3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine zwitterion+proton4-(trimethylamino)butanal+glycine
Catalysed by SHMT2
Reaction pageLeft to rightBalancedEC 2.1.2.1mitochondrial matrix
Human-GEM MAR01371CC BY 4.0Reference data
(6R)-5,10-methenyltetrahydrofolate+waterMAM01100m+proton
Catalysed by SHMT2
Reaction pageLeft to rightBalancedEC 2.1.2.10mitochondrial matrix
3 names
Resolves to: SHMT2
(6R)-5,10-methylenetetrahydrofolic acid+glycine+waterL-seryl residue+5,6,7,8-tetrahydrofolic acid
Catalysed by SHMT2
Reaction pageReversibleBalancedEC 2.1.2.1mitochondrial matrix
Human-GEM MAR04792CC BY 4.0Reference data
(6R)-5,10-methylene-5,6,7,8-tetrahydrofolate+glycine+water(6S)-5,6,7,8-tetrahydrofolate+L-seryl residue
Reaction pageReversibleBalanced
Rhea RHEA:15481CC BY 4.0Reference data