EC Number |
Protein Variants |
Reference |
---|
2.8.3.5 | A215V |
the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency |
738807 |
2.8.3.5 | A215V |
the mutation is associated with succinyl-CoA:3-ketoacid CoA transferase deficiency and retains 3.5% residual activity |
724424 |
2.8.3.5 | C129S |
like wild-type SCOT rapid modification in the presence of acyl-CoA substrates |
672216 |
2.8.3.5 | C196S |
site-directed mutagenesis, the mutant protein is modified rapidly in the presence of acyl-CoA substrates in analogy to the wild-type enzyme |
672216 |
2.8.3.5 | C196S |
specific activity similar to wild-type SCOT |
672216 |
2.8.3.5 | C28A |
specific activity similar to C28S mutant |
672216 |
2.8.3.5 | C28S |
site-directed mutagenesis, the mutant protein is modified much more slowly than the wild-type enzyme, indicating that Cys28 is the residue exposed on binding CoA, the specific activity of the C28S mutant protein is unexpectedly lower than that of wild-type SCOT |
672216 |
2.8.3.5 | C28S |
slow modification in the presence of acyl-CoA substrates, a chloride ion is bound to one of four active sites in the crystal structure of the C28S mutant protein, mimicking substrate, interacting with Lys329, Asn51, and Asn52 |
672216 |
2.8.3.5 | C456F |
missense mutation derived from a SCOT-deficient patient, no enzyme activity |
645992 |
2.8.3.5 | C456F |
no activity |
663018 |