GGT1: Initiates extracellular glutathione (GSH) breakdown, provides cells with a local cysteine supply and contributes to maintain intracellular GSH level. It is part of the cell antioxidant defense mechanism. Catalyzes the transfer of the glutamyl moiety of glutathione to amino acids and dipeptide acceptors. Alternatively, glutathione can be hydrolyzed to give Cys-Gly and gamma glutamate. Isoform 3 seems to be inactive. Defects in GGT1 are a cause of glutathionuria (GLUTH); also known as gamma-glutamyltranspeptidase deficiency. It is an autosomal recessive disease. Belongs to the gamma-glutamyltransferase family. 3 isoforms of the human protein are produced by alternative promoter.
Protein type: EC 2.3.2.2; EC 3.4.19.13; EC 3.4.19.14; Lipid Metabolism - arachidonic acid; Membrane protein, integral; Other Amino Acids Metabolism - cyanoamino acid; Other Amino Acids Metabolism - glutathione; Other Amino Acids Metabolism - selenoamino acid; Other Amino Acids Metabolism - taurine and hypotaurine; Transferase
Chromosomal Location of Human Ortholog: 10|10 C1
Cellular Component: extracellular space; integral component of membrane; integral component of plasma membrane; membrane; plasma membrane; vesicle
Molecular Function: hydrolase activity; peptidase activity; peptidyltransferase activity; transferase activity; transferase activity, transferring acyl groups; transferase activity, transferring amino-acyl groups
Biological Process: amino acid metabolic process; cysteine biosynthetic process; fatty acid metabolic process; glutamate metabolic process; glutathione biosynthetic process; glutathione catabolic process; glutathione metabolic process; peptide modification; proteolysis; regulation of immune system process; regulation of inflammatory response; spermatogenesis; zymogen activation
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