| Autor: |
E G, van Grunsven, E, van Berkel, S, Denis, P A, Mooijer, R J, Wanders |
| Rok vydání: |
2000 |
| Předmět: |
|
| Zdroj: |
Advances in experimental medicine and biology. 466 |
| ISSN: |
0065-2598 |
| Popis: |
The second and third steps of peroxisomal beta-oxidation are catalysed by two multifunctional enzymes: D-bifunctional protein and L-bifunctional protein. Here we show that fibroblasts of a patient described as being deficient in the 3-hydroxyacyl-CoA dehydrogenase component of D-bifunctional protein and fibroblasts of a patient described as being deficient in L-bifunctional protein do not complement one another. Using a newly developed method to measure the activity of D-bifunctional protein in fibroblast homogenates, we found that the activity of the D-bifunctional protein was completely deficient in the patient with presumed L-bifunctional protein deficiency. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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