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Literature summary for 6.3.2.2 extracted from

  • Foeller, M.; Harris, I.S.; Elia, A.; John, R.; Lang, F.; Kavanagh, T.J.; Mak, T.W.
    Functional significance of glutamate-cysteine ligase modifier for erythrocyte survival in vitro and in vivo (2013), Cell Death Differ., 20, 1350-1358.
    View publication on PubMedView publication on EuropePMC
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + L-glutamate + L-cysteine Mus musculus
-
 ADP + phosphate + gamma-L-glutamyl-L-cysteine
-
 ?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
erythrocyte
-
 Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + L-glutamate + L-cysteine
-
 Mus musculus ADP + phosphate + gamma-L-glutamyl-L-cysteine
-
 ?

Subunits

Subunits Comment Organism
heterodimer glutamate-cysteine ligase consists of a catalytic subunit (GCLC) and a modifier subunit (GCLM) Mus musculus

Cofactor

Cofactor Comment Organism Structure
ATP
-
 Mus musculus

General Information

General Information Comment Organism
malfunction erythrocytes from gclm-/- mice show greatly reduced intracellular glutathione. Prolonged incubation results in complete lysis of gclm-/- erythrocytes, which can be reversed by exogenous delivery of the antioxidant Trolox. Phenylhydrazine-induced oxidative stress in glcm-/- causes dramatically increased hemolysis, markedly larger accumulations of injured erythrocytes in the spleen, erythrocyte-derived pigment hemosiderin in kidney tubules, and diminished kidney function compared to wild-type mice, phenotype, overview. Regulatory subunit GCLM-deficient erythrocytes are more prone to Ca2+-dependent suicidal cell death ex vivo. Without additional oxidative stress, the mutant animals are able to survive by slightly ramping up their generation of new erythrocytes Mus musculus
physiological function the enzyme is rate-limiting for glutathione synthesis Mus musculus