Wanders R.J.A.: Focus of research

Peroxisomes were long thought to play only a minimal role in human physiology until the early 1980s when studies in a rare lethal autosomal recessive disorder called Zellweger syndrome revealed that peroxisomes are in fact indispensible organelles playing a key role in human physiology. Indeed, peroxisomes are now known to catalyze multiple metabolic functions including: (1.) the beta-oxidation of certain fatty acids notably very long-chain fatty acids; (2.) the synthesis of etherphospholipids (plasmalogens); (3.) the alpha-oxidation of 3-methyl branched chain fatty acids including phytanic acid, and (4.) the detoxification of glyoxylate. Subsequent to the discovery of Zellweger syndrome as the prototype peroxisomal disorders many additional peroxisomal diseases have been identified. .......