The genetics of peroxisome biogenesis

The segregation of metabolic functions within discrete organelles is a hall mark of eukaryotic cells. These compartments allow for the concentration of related metabolic functions, the separation of competing metabolic functio ns, and the formation of unique chemical microenvironments. However, such o rganization is not spontaneous and requires an array of genes that are dedi cated to the assembly and maintenance of these structures. In this review w e focus on the genetics of peroxisome biogenesis and on how defects in this process cause human disease.