PEROXISOMAL DISORDERS - OXYGEN AND FREE-R ADICALS

Peroxisomes were the latest subcellular organelles to be discovered. F or a long time, due to limited knowledge of their function, peroxisome s were considered to play only a supporting role for other organelles in cellular metabolism. The discovery of specific metabolic pathways c arried out exclusively in peroxisomes and, in particular, the descript ion of genetically determined peroxisomal disorders such as the Zellwe ger syndrome and X-linked adrenoleukodystrophy, established the import ance of these organelles in playing an essential role in cellular meta bolism. Recently, their participation in the metabolism of reactive ox ygen species and free radicals was found to be one of the important fu nctions of peroxisomes. The fact that peroxisomal disorders are well d efined biochemically and that peroxisomes consume a significant amount of the total cellular oxygen uptake, make cells from patients sufferi ng from these disorders a suitable cell model for studying peroxisomal participation in the detoxification of reactive oxygen species.