Glutathione synthetase

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glutathione synthetase
Identifiers
Symbol GSS
Entrez 2937
HUGO 4624
OMIM 601002
RefSeq NM_000178
UniProt P48637
Other data
Locus Chr. 20 q11.2
Eukaryotic glutathione synthase
PDB 2hgs EBI.jpg
human glutathione synthetase
Identifiers
Symbol GSH_synthase
Pfam PF03199
Pfam clan CL0483
InterPro IPR004887
SCOP 2hgs
SUPERFAMILY 2hgs
Eukaryotic glutathione synthase, ATP binding domain
PDB 2hgs EBI.jpg
human glutathione synthetase
Identifiers
Symbol GSH_synth_ATP
Pfam PF03917
InterPro IPR005615
SCOP 1m0t
SUPERFAMILY 1m0t
Prokaryotic glutathione synthetase, N-terminal domain
PDB 1gsh EBI.jpg
structure of escherichia coli glutathione synthetase at ph 7.5
Identifiers
Symbol GSH-S_N
Pfam PF02951
InterPro IPR004215
SCOP 1glv
SUPERFAMILY 1glv
Prokaryotic glutathione synthetase, ATP-grasp domain
PDB 1gsh EBI.jpg
structure of escherichia coli glutathione synthetase at ph 7.5
Identifiers
Symbol GSH-S_ATP
Pfam PF02955
Pfam clan CL0179
InterPro IPR004218
SCOP 1glv
SUPERFAMILY 1glv

Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione.[1]

In eukaryotes, this is a homodimeric enzyme. In humans, defects in GSS are inherited in an autosomal recessive way and are the cause of severe metabolic acidosis, 5-oxoprolinuria, and increased rate of haemolysis and defective function of the central nervous system. The substrate-binding domain has a 3-layer alpha/beta/alpha structure.[2]

See also[edit]

References[edit]

  1. ^ Njålsson R, Norgren S (2005). "Physiological and pathological aspects of GSH metabolism.". Acta Paediatr 94 (2): 132–7. doi:10.1080/08035250410025285. PMID 15981742. 
  2. ^ Polekhina G, Board PG, Gali RR, Rossjohn J, Parker MW (June 1999). "Molecular basis of glutathione synthetase deficiency and a rare gene permutation event". EMBO J. 18 (12): 3204–13. doi:10.1093/emboj/18.12.3204. PMC 1171401. PMID 10369661. 

External links[edit]