NCIt 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);