Glutamate-cysteine ligase, also known as gamma-glutamylcysteine synthetase, is the first rate limiting enzyme of glutathione synthesis. The enzyme consists of two subunits, a heavy catalytic subunit and a light regulatory subunit. Gamma glutamylcysteine synthetase deficiency has been implicated in some forms of hemolytic anemia. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Apr 2015],pathway:Sulfur metabolism, glutathione biosynthesis, glutathione from L-cysteine and L-glutamate: step 1/2.,similarity:Belongs to the aldo/keto reductase family. Glutamate--cysteine ligase light chain subfamily.,subunit:Heterodimer of a catalytic heavy chain and a regulatory light chain.,tissue specificity:In all tissues examined. Highest levels in skeletal muscle.,
