Virchows Archiv. A, Pathological anatomy and histopathology 1991
Very large peroxisomes in distinct peroxisomal disorders (rhizomelic chondrodysplasia punctata and acyl-CoA oxidase deficiency): novel data.
Tylko zarejestrowani użytkownicy mogą tłumaczyć artykuły
Zaloguj się Zarejestruj się
Link zostanie zapisany w schowku
Słowa kluczowe
Abstrakcyjny
We report very large hepatic peroxisomes (d-circle greater than 1 micron) in a patient with rhizomelic chondrodysplasia punctata and a patient with acyl-CoA oxidase deficiency. The effects of peroxisomal enlargement on the enzymatic activity are discussed. As increase in peroxisomal size is also reported in at least 12 other patients with peroxisomal disorders, we propose a relationship between the enlargement of the organelles and their functional deficiency.
