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This review is the first one dealing specifically with a class of peroxisomal disorders that remain virtually unfamiliar to Russian medical genetic consultants and pediatricians. Data are presented that concern classification, genetics, characterization of clinical and biochemical phenotype, prevention, and therapy of 18 nosological units included in the class of peroxisomal disorders. Problems of general biological significance are reviewed, which can be solved using peroxisomal disorders as a valuable experimental model.

Yeast peroxisomes (microbodies) were studied, particularly with yeast methylotrophs, using light-optical microscopy, viz. intravitam phase- and anoptral-contrast microscopy, with cytochemical as well as fixed and stained objects. The data obtained were compared with cytochemical as well as fixed and stained objects. The data obtained were compared with other evidence that we had gained on methylotrophs studied by electron microscopy and with the results obtained by investigating the peroxisomes of yeast cultures grown on nutrient containing other carbon sources. The activity of exocatalase and endocatalase, the enzymes characteristic of peroxisomes, was assayed at different growth phases of yeast methylotrophs and was found to be correlated with the cycle of development of peroxisomes. The content of biotin and inositol and its dynamics were studied in the methylotroph cells. Possible origin of peroxisomes is discussed as well as their development and the mechanism of degradation. A close contact and functional relationship have been established between peroxisomes and mitochondria.