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Although peroxisomes were initially believed to play only a minor role in mammalian metabolism, it is now clear that they catalyse essential reactions in a number of different metabolic pathways and thus play an indispensable role in intermediary metabolism. The metabolic pathways in which
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
Prostaglandin E2 synthesis and eicosanoid biosynthetic enzyme activities (arachidonyl CoA synthetase, cyclooxygenase and phospholipase A2) were measured in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin and compared to those from normal subjects and patients with
METHODS
Peroxisomal deficiency has been described in a number of syndromes characterized by chondrodysplasia punctata, including the Conradi-Hünermann (C-H) syndrome. Because of overlapping clinical features of X-chromosome inheritance, ichthyosis, and limb-reduction defects in C-H and CHILD
Peroxisomes are ubiquitous subcellular organelles varying in number, size and enzymatic content according to species, tissues or physiological states. Microperoxisomes are present in the central nervous system and in muscle. Peroxisomes participate in anabolic and catabolic processes, including