glutamate-cysteine ligase catalytic subunit; gamma-glutamylcysteine synthetase catalytic subunit (GCLC)

Function

• sulfur metabolism; glutathione biosynthesis; glutathione from L-cysteine & L-glutamate: step 1/2
• feedback inhibition by glutathione

ATP + L-glutamate + L-cysteine
        <-->
ADP + phosphate + gamma-L-glutamyl-L-cysteine

Structure

• heterodimer of a catalytic heavy chain & a regulatory light chain
• belongs to the glutamate-cysteine ligase type 3 family

Pathology

defects in GCLC are the cause of hemolytic anemia

More general terms

• enzyme catalytic subunit

Component of

• glutamate-cysteine ligase; gamma-glutamylcysteine synthetase

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2729
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2729
• OMIM: https://mirror.omim.org/entry/230450
• OMIM: https://mirror.omim.org/entry/606857
• UniProt: http://www.uniprot.org/uniprot/P48506.html