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Literature summary for 6.3.2.2 extracted from

  • Willis, M.N.; Liu, Y.; Biterova, E.I.; Simpson, M.A.; Kim, H.; Lee, J.; Barycki, J.J.
    Enzymatic defects underlying hereditary glutamate cysteine ligase deficiency are mitigated by association of the catalytic and regulatory subunits (2011), Biochemistry, 50, 6508-6517.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 6.3.2.2

Cloned(Commentary)

Cloned (Comment) Organism
transfecttion of embryonic fibroblast from GCLC null mice and expression in Saccharomyces cerevisiae Homo sapiens

Protein Variants

Protein Variants Comment Organism
H370L clinically relevant mutation in catalytic subunit GCLC. Significantly lower levels of glutathione relative to that of the wild type. Compromised enzymatic activity can largely be rescued by the addition of GCLM Homo sapiens
P158L clinically relevant mutation in catalytic subunit GCLC. Significantly lower levels of glutathione relative to that of the wild type, kinetic constants comparable to those of wild-type GCLC Homo sapiens
P414L clinically relevant mutation in catalytic subunit GCLC. Significantly lower levels of glutathione relative to that of the wild type, most compromised mutant among those studied. Compromised enzymatic activity can largely be rescued by the addition of GCLM Homo sapiens
R127C clinically relevant mutation in catalytic subunit GCLC. Significantly lower levels of glutathione relative to that of the wild type. Compromised enzymatic activity can largely be rescued by the addition of GCLM Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.05
-
 L-Cys mutant R127C, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.07
-
 L-Cys wild-type, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.08
-
 L-Cys mutant P138L, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.1
-
 L-Cys wild-type, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.12
-
 L-Cys mutant R127C, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.16
-
 L-Cys mutant H370L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.17
-
 L-Cys mutant P138L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.17
-
 L-Cys mutant P414L, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.26
-
 ATP mutant H370L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.3
-
 ATP mutant P138L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.44
-
 L-Glu mutant H370L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.44
-
 ATP wild-type, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.46
-
 L-Glu wild-type, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.5
-
 ATP mutant R127C, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.65
-
 L-Glu mutant P138L, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
0.68
-
 L-Glu mutant P138L, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.77
-
 L-Glu mutant P414L, catalytic subunit, pH 8.0, 37°C Homo sapiens
0.82
-
 ATP mutant P414L, catalytic subunit, pH 8.0, 37°C Homo sapiens
1.14
-
 L-Glu wild-type, catalytic subunit, pH 8.0, 37°C Homo sapiens
1.38
-
 L-Glu mutant R127C, catalytic subunit, pH 8.0, 37°C Homo sapiens
1.38
-
 L-Glu mutant R127C, CGL holoenzyme, pH 8.0, 37°C Homo sapiens
2.68
-
 ATP wild-type, catalytic subunit, pH 8.0, 37°C Homo sapiens
3.57
-
 ATP mutant P138L, catalytic subunit, pH 8.0, 37°C Homo sapiens
4.47
-
 ATP mutant R127C, catalytic subunit, pH 8.0, 37°C Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + L-Glu + L-Cys
-
 Homo sapiens ADP + phosphate + gamma-L-Glu-L-Cys
-
 ?

Synonyms

Synonyms Comment Organism
GCLC GCL catalytic subunit Homo sapiens