Cyclooxygenase pathway in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin.
Cyclooxygenase metabolism was studied in fibroblasts from patients with metabolic disorders of peroxisomal origin (adrenomyeloneuropathy, X-linked adrenoleukodystrophy, cerebrohepatorenal syndrome of Zellweger and rhizomelic chondrodysplasia punctata). In response to arachidonic acid (6.25-100 microM) or calcium ionophore A23187 (2.5-20 microM) prostaglandin E2 and 6-keto-prostaglandin F1 alpha are the main cyclooxygenase metabolites formed. No formation of thromboxane B2 or 2,3-dinor-thromboxane B2 was found. Apparently due to the heterogeneous nature of peroxisomal disorders no uniform pattern of cyclooxygenase metabolism and eicosanoid concentrations in cell lines from patients with peroxisomal defects was found.
Malle
E
E
Philipps University, Department of Internal Medicine, Marburg/Lahn, Germany.
Leis
H J
HJ
Steinmetz
A
A
Paschke
E
E
Hoefler
G
G
D015121:6-Ketoprostaglandin F1 alpha / Q000096:biosynthesis; D000326:Adrenoleukodystrophy / Q000201:enzymology* / Q000235:genetics; D002478:Cells, Cultured; D002806:Chondrodysplasia Punctata / Q000201:enzymology*; D015232:Dinoprostone / Q000096:biosynthesis; D005347:Fibroblasts / Q000201:enzymology; D008040:Genetic Linkage; D006801:Humans; D008830:Microbodies / Q000378:metabolism*; D011451:Prostaglandin-Endoperoxide Synthases / Q000378:metabolism*; D012867:Skin / Q000166:cytology / Q000201:enzymology*; D014960:X Chromosome; D015211:Zellweger Syndrome / Q000201:enzymology*
D015121:6-Ketoprostaglandin F1 alpha; D011451:Prostaglandin-Endoperoxide Synthases; D015232:Dinoprostone