Cloned (Comment) | Organism |
---|---|
expression of wild-type and mutants in Escherichia coli | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
170000 | - |
gel filtration | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
isovaleryl-CoA + acceptor | Homo sapiens | - |
3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
additional information | Homo sapiens | the type III enzyme mutation responsible for isovaleric acidemia leads to a shift in reading frame and the subsequent incorporation of eight abnormally placed amino acids with premature termination of translation | ? | - |
? | |
additional information | Homo sapiens | a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia | ? | - |
? | |
additional information | Homo sapiens | the enzyme catalyzes the third step of the leucine oxidative metabolism | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
0.0041 | - |
normal cell lines | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
isovaleryl-CoA + acceptor | - |
Homo sapiens | 3-methylcrotonyl-CoA + reduced acceptor | - |
? | |
additional information | the type III enzyme mutation responsible for isovaleric acidemia leads to a shift in reading frame and the subsequent incorporation of eight abnormally placed amino acids with premature termination of translation | Homo sapiens | ? | - |
? | |
additional information | a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia | Homo sapiens | ? | - |
? | |
additional information | the enzyme catalyzes the third step of the leucine oxidative metabolism | Homo sapiens | ? | - |
? |