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glutathione [GSH] synthetase (GSS)
Function:
- sulfur metabolism; glutathione biosynthesis;
- glutathione from L-cysteine & L-glutamate: step 2/2
ATP + gamma-L-glutamyl-L-cysteine + glycine
ADP + phosphate + glutathione
Structure:
- homodimer (putative)
- belongs to the eukaryotic GSH synthase family
Pathology:
- defects in GSS are the cause of glutathione synthetase deficiency
Interactions
molecular events
Related
gamma glutamyl cycle
glutathione; gamma-glutamylcysteinylglycine (GSH)
General
ligase or synthetase
Properties
SIZE: entity length = 474 aa
MW = 52 kD
MOTIF: active site
Database Correlations
OMIM correlations
MORBIDMAP 601002
UniProt P48637
PFAM correlations
KEGG correlations
ENZYME 6.3.2.3
References
- Textbook of Biochemistry with Clinical Correlations,
3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 524
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=GSS
- UniProt :accession P48637