Application | Comment | Organism |
---|---|---|
medicine | the developed LC-MS/MS method is able to provide ample sensitivity to correctly and rapidly determine MCAD and VLCAD residual activity in human lymphocytes | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A985G | nearly all patients with residual MCAD activities below 2.5% are homozygous 985A>G carriers | Homo sapiens |
T199C | all patients with the 199T>C mutation have residual activities above 10% | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
lymphocyte | - |
Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
additional information | - |
heterozygous ACADVL mutation carriers show residual VLCAD activities of 23.7 to 54.2% | Homo sapiens |
additional information | - |
residual VLCAD activity of patients with confirmed VLCAD-deficiency is between 0.3 and 1.1% | Homo sapiens |
0.00238 | - |
MCAD activity, controls | Homo sapiens |
0.00695 | - |
VLCAD activity, controls, range 0.00195 to 0.01191 | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
octanoyl-CoA + acceptor | MCAD activity assay | Homo sapiens | ? | - |
? | |
palmitoleoyl-CoA + acceptor | VLCAD activity assay | Homo sapiens | ? | - |
? | |
palmitoyl-CoA + acceptor | VLCAD activity assay | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
LCAD | - |
Homo sapiens |
long-chain acyl-CoA dehydrogenase | - |
Homo sapiens |
MCAD | - |
Homo sapiens |
medium long-chain acyl-CoA dehydrogenase | - |
Homo sapiens |
very long-chain acyl-CoA dehydrogenase | - |
Homo sapiens |
VLCAD | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
activity assay | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
8 | - |
activity assay | Homo sapiens |