ENTRY EC 6.3.2.2
NAME Glutamate--cysteine ligase
gamma-Glutamylcysteine synthetase
CLASS Ligases
Forming carbon-nitrogen bonds
Acid--amino-acid ligases (peptide synthases)
SYSNAME L-Glutamate:L-cysteine gamma-ligase (ADP-forming)
REACTION ATP + L-Glutamate + L-Cysteine = ADP + Orthophosphate +
gamma-L-Glutamyl-L-cysteine
SUBSTRATE ATP
L-Glutamate
L-Cysteine
L-Aminohexanoate
PRODUCT ADP
Orthophosphate
gamma-L-Glutamyl-L-cysteine
INHIBITOR S-Butyl-DL-homocysteine-[S,R]-sulfoximine
COMMENT Can use L-aminohexanoate in place of glutamate.
PATHWAY PATH: MAP00251 Glutamate metabolism
PATH: MAP00480 Glutathione metabolism
DISEASE MIM: 230450 Glutamate-cysteine ligase (gamma-glutamylcysteine
synthetase),; Hemolytic anemia due to
gamma-glutamylcysteine synthetase deficiency (1)
MIM: 601176 Glutamate-cysteine ligase (gamma-glutamylcysteine
synthetase),
GENES ECO: b2688(gshA)
SCE: YJL101C(GSH1)
HSA: GLCLC(GCS) GLCLR(Hs.89709)
DBLINKS University of Geneva ENZYME DATA BANK: 6.3.2.2
WIT (What Is There) Metabolic Reconstruction: 6.3.2.2
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