Gamma-Glutamylcysteine Synthetase

**Semantic type:** Amino Acid, Peptide, or Protein|Enzyme

**Definition:** 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. The gene encoding the catalytic subunit encodes a protein of 367 amino acids with a calculated molecular weight of 72.773 kDa and maps to chromosome 6. The regulatory subunit is derived from a different gene located on chromosome 1p22-p21. Deficiency of gamma-glutamylcysteine synthetase in human is associated with enzymopathic hemolytic anemia. (LocusLink)

**Synonyms:** - EC 6.3.2.2 - Gamma-Glutamylcysteinyl Synthetase - Glutamate-Cysteine Ligase