EC Number   |
Application |
Reference |
|---|
    6.4.1.3 | analysis |
method to analyze propionyl-CoA carboxylase activity in phytohemagglutinin stimulated lymphocytes using high performance liquid chromatography. Propionyl-CoA carboxylase activity is unaffected even when lymphocytes are isolated and phytohemagglutinin stimulated after a whole blood sample has been stored at 4°C for 5 days, and the method is useful for the confirmation of propionic acidemia in individuals, and prenatal diagnosis and genetic counseling for the affected families |
744741 |
| 6.4.1.3 | medicine |
living-donor liver transplantation is an effective treatment modality in patients with congenial metabolic liver disease, i. e., propionic acidemia caused by deficiency in propionyl-CoA carboxylase |
706079 |
| 6.4.1.3 | medicine |
propionyl-CoA carboxylase is a sensitive indicator of biotin status |
671196 |
| 6.4.1.3 | medicine |
stringent model of propionyl CoA carboxylase subunit A deficiency, where homozygous knock-out mice are born, but die within 36 hours. Injection of vectors expressing propionyl CoA carboxylase subunit A significantly increases the lifespan for both unmodified and polyethylene glycol modified vectors, but the rescue is transient |
692572 |
| 6.4.1.3 | medicine |
treatment of propionic acidemia by import of a biologically active, fully assembled propionyl-CoA carboxylase dodecamer, posttranslationally chemically conjugated with the HIV TAT peptide. TAT-propionyl-CoA carboxylase is transferred into isolated mitochondria, as well as into mitochondria of patient fibroblasts . A single-dose intraperitoneal injection into propionyl-CoA carboxylase-deficient mice decreases the propionylcarnitine/acetylcarnitine (C3/C2) ratio toward the normal level |
745766 |
| 6.4.1.3 | synthesis |
expression of the propionyl-CoA carboxylase complex from Streptomyces coelicolor supports the highest levels of heterologous polyketide production in Escherichia coli. The molar yield of 6-deoxyerythronolide B of Escherichia coli, harboring the wild-type Streptomyces coelicolor propionyl-CoA carboxylase, is 1.2% |
745187 |
