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Literature summary for 4.4.1.1 extracted from

  • Zhu, W.; Lin, A.; Banerjee, R.
    Kinetic properties of polymorphic variants and pathogenic mutants in human cystathionine gamma-lyase (2008), Biochemistry, 47, 6226-6232.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine high doses of pyridoxine could be an effective therapy for cystathioninuric patients harboring the T67I mutation and be somewhat less effective in ameliorating the symptoms associated with the Q240E mutation Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
Q240E missense mutation. Exhibits a 70fold decrease in Vmax compared to that of wild-type CGL. The KMs for L-cystathionine are comparable to that of wild type CGL. The pyridoxal 5'-phosphate content of the Q240E mutant is about 80fold lower than that of wild-type enzyme Homo sapiens
T67I missense mutation. Exhibits a 3.5fold decrease in Vmax compared to that of wild-type CGL. The KMs for L-cystathionine are comparable to that of wild type CGL. The pyridoxal 5'-phosphate content of the T67I mutant is about 4fold lower than that of wild-type enzyme Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.4
-
L-cystathionine for variant S403 Homo sapiens
0.6
-
L-cystathionine for mutant T67I Homo sapiens
0.6
-
L-cystathionine for variant I403 Homo sapiens
0.72
-
L-cystathionine for mutant Q240E Homo sapiens
3.4
-
L-cysteine for variant I403 Homo sapiens
3.5
-
L-cysteine for variant S403 Homo sapiens
4.1
-
L-cysteine for mutant T67I Homo sapiens
5.4
-
L-homocysteine for variant S403 Homo sapiens
7
-
L-homocysteine for variant I403 Homo sapiens
8
-
L-homocysteine for mutant T67I Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
156000
-
T67I CGL mutant Homo sapiens
156000
-
wild-type, estimated for CGL from its retention time of 42.8 min Homo sapiens
170000
-
Q240E CGL mutant Homo sapiens
178000
-
wild-type, molecular masses of both the T67I CGL (156 kDa) and the Q240E mutant (170 kDa) are comparable to that of wild-type CGL (167 kDa), indicating that both mutants exist as tetrameric proteins Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P32929
-
-

Purification (Commentary)

Purification (Comment) Organism
combination of Ni-NTA chromatography, anion exchange chromatography, and gel filtration steps Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-cystathionine + H2O
-
Homo sapiens L-cysteine + 2-oxobutanoate + NH3
-
?
L-cysteine + H2O
-
Homo sapiens sulfide + NH3 + pyruvate
-
?
L-homocysteine
-
Homo sapiens ?
-
?

Subunits

Subunits Comment Organism
homotetramer In the three-dimensional structure of human CGL, two monomers contribute residues to each of the two active sites in the dimer, and two dimers come together to form a tetramer Homo sapiens

Synonyms

Synonyms Comment Organism
cgl CGL variant S403 and I403, polymorphic variants at amino acid residues 403 Homo sapiens
human cystathionine-gamma-lyase pyridoxal-5'-phosphate (PLP)-dependent enzyme Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
0.02
-
L-cystathionine for mutante Q240E Homo sapiens
0.3
-
L-cysteine for mutante T67I Homo sapiens
0.45
-
L-cystathionine for mutante T67I Homo sapiens
0.67
-
L-cysteine for variante I403 Homo sapiens
0.67
-
L-cysteine for variante S403 Homo sapiens
0.82
-
L-homocysteine for mutante T67I Homo sapiens
1.7
-
L-cystathionine for variante S403 Homo sapiens
1.9
-
L-cystathionine for variante I403 Homo sapiens
3.5
-
L-homocysteine for variante S403 Homo sapiens
3.7
-
L-homocysteine for variante I403 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
pyridoxal 5'-phosphate
-
Homo sapiens