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Proteins/UNIPROT:P23434
The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST). Has a pivotal role in the lipoylation of enzymes involved in cellular energetics such as the mitochondrial dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex (DLAT), and the mitochondrial dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex (DLST)..
Function from UniProtKB (P23434), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 1 · substrate of
1 compound
1 compound.
0 reported routes1 assembled route
4 rows
16 statements
16 statements · 5 reference databases · Subject: GCSH · Sources: Hetionet, Human-GEM, Reactome, UniProt, WikiPathways
4 reactions
MAM02643mMAM00210m+MAM02484m
Catalysed by LIPT2 & NDUFAB1 & GCSH
Reaction pageLeft to rightBalance undeterminableEC 2.3.1.181mitochondrial matrix
Human-GEM MAR06402CC BY 4.0Reference data
MAM00210m+3proton+NADPH(4-)+2S-adenosyl-L-methionine+tetra-mu3-sulfido-tetrairon(2+)MAM00208m+2MAM01098m+
2 names
Resolves to: GCSH
Catalysed by LIAS & FDX1 & FDXR & GCSH & ISCA1 or LIAS & FDX1 & FDXR & GCSH & ISCA2 or LIAS & FDX1 & FDXR & GCSH & NFU1
Reaction pageLeft to rightBalancedEC 2.8.1.8mitochondrial matrix
Human-GEM MAR06403CC BY 4.0Reference data
MAM00209m+glycine+protoncarbon dioxide+S(8)-aminomethyldihydrolipoamide
Reaction pageLeft to rightUnbalancedEC 1.8.1.4, 2.1.2.10mitochondrial matrix
Human-GEM MAR08433CC BY 4.0Reference data
S(8)-aminomethyldihydrolipoamide+5,6,7,8-tetrahydrofolic acidMAM00208m+(6R)-5,10-methylenetetrahydrofolic acid+proton+ammonium
Reaction pageLeft to rightUnbalancedEC 1.8.1.4, 2.1.2.10mitochondrial matrix
Human-GEM MAR08434CC BY 4.0Reference data