Peroxisomes

Peroxisomes are important cell organelles of a very simple architecture that display a wide repertoire of metaboLic functions. In man, over 50 peroxisomal enzymes are known. Major peroxisomal metaboLic pathways are α- and β-oxidation of fatty acids, ether Lipid and bile acid biosynthesis, and the metaboLism of glyoxylate, purines, polyamines, and d-amino acids. As yet, two types of peroxisomal disorders have been identified, namely, those in which the formation of the organelles is disturbed or in which a single peroxisomal enzyme or transporter is defective. Generally, in these patients, substrates that are degraded by peroxisomal enzymes accumulate, or compounds that are synthesized in these organelles are reduced. This causes a variety of cLinical symptoms, which are often very severe resulting in an early death.