Peroxisomes are organelles that confine an important set of enzymes within
their single membrane boundaries. In man, a wide variety of genetic disorde
rs is caused by loss of peroxisome function. In the most severe cases, the
clinical phenotype indicates that abnormalities begin to appear during embr
yological development. In less severe cases, the quality of life of adults
is affected. Research on yeast model systems has contributed to a better un
derstanding of peroxisome formation and maintenance. This framework of know
ledge has made it possible to understand the molecular basis of most of the
peroxisome biogenesis disorders. Interestingly, most peroxisome biogenesis
disorders are caused by a failure to target peroxisomal proteins to the or
ganellar matrix or membrane, which classifies them as protein targeting dis
eases. Here we review recent fundamental research on peroxisomal protein ta
rgeting and discuss a few burning questions in the field concerning the ori
gin of peroxisomes. (C) 1999 Elsevier Science B.V. All rights reserved.