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Literature summary for 2.3.1.9 extracted from

  • Sakurai, S.; Fukao, T.; Haapalainen, A.M.; Zhang, G.; Yamada, K.; Lilliu, F.; Yano, S.; Robinson, P.; Gibson, M.K.; Wanders, R.J.; Mitchell, G.A.; Wierenga, R.K.; Kondo, N.
    Kinetic and expression analyses of seven novel mutations in mitochondrial acetoacetyl-CoA thiolase (T2): Identification of a K(m) mutant and an analysis of the mutational sites in the structure (2007), Mol. Genet. Metab., 90, 370-378.
    View publication on PubMed

Protein Variants

Protein Variants Comment Organism
E252del relative protein amount and enzyme activity of 30% and 25% respectively, in comparison to the wild-type at 37°C. 2fold Km-elevation for substrates coenzyme A and acetoacetyl-CoA compared to wild-type values. Vmax is comparable to wild-type value Homo sapiens
E252del 25% of wild-type activity at 37°C, 40% of wild-type activity at 30°C. Mutant is unstable compared to the wild-type protein at 37°C. KM-value for acetoacetyl-CoA is 2fold higher than wild-type value. Km-value for CoA is 1.8fold lower than wild-type value Homo sapiens
E252del no residual activity under any condition Homo sapiens
N158D inactive mutant protein Homo sapiens
N158D no residual activity under any condition Homo sapiens
N158S inactive mutant protein Homo sapiens
N158S no residual activity under any condition Homo sapiens
N282H inactive mutant protein Homo sapiens
N282H no residual activity under any condition Homo sapiens
N353K cDNAs yield neither residual T2 protein nor enzyme activity Homo sapiens
N353K no residual activity under any condition Homo sapiens
Q272X no residual activity under any condition Homo sapiens
Q73P cDNAs yield neither residual T2 protein nor enzyme activity Homo sapiens
Q73P no residual activity under any condition Homo sapiens
R208Q inactive mutant protein Homo sapiens
R208Q no residual activity under any condition Homo sapiens
Y219H inactive mutant protein Homo sapiens
Y219H no residual activity under any condition Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
acetoacetyl-CoA substrate inhibition beyond 0.0225 mM Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.005
-
acetoacetyl-CoA wild-type enzyme Homo sapiens
0.005
-
acetoacetyl-CoA E252del mutant enzyme Homo sapiens
0.01
-
acetoacetyl-CoA wild-type enzyme Homo sapiens
0.01
-
acetoacetyl-CoA mutant enzyme E252del Homo sapiens
0.028
-
CoA wild-type enzyme Homo sapiens
0.05
-
CoA mutant enzyme E252del Homo sapiens
0.05
-
CoA E252del mutant enzyme Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Homo sapiens 5739
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
acetyl-CoA + acetyl-CoA
-
Homo sapiens CoA + acetoacetyl-CoA
-
?
CoA + acetoacetyl-CoA
-
Homo sapiens acetyl-CoA + acetyl-CoA
-
?

Synonyms

Synonyms Comment Organism
acetoacetyl-CoA thiolase T2
-
Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
37
-
wild-type T2 protein is stable even after chasing for 48 h at 37°C. The amount of E252del mutant protein at 48 h incubation with cycloheximide is estimated to be 50% of that observed at 0 h. E252del mutant T2 is unstable compared to the wild-type protein at 37°C Homo sapiens