Peroxisomes, lipid metabolism and lipotoxicity.
Peroxisomes play an essential role in cellular lipid metabolism as exemplified by the existence of a number of genetic diseases in humans caused by the impaired function of one of the peroxisomal enzymes involved in lipid metabolism. Key pathways in which peroxisomes are involved include: (1.) fatty acid beta-oxidation; (2.) etherphospholipid biosynthesis, and (3.) fatty acid alpha-oxidation. In this paper we will describe these different pathways in some detail and will provide an overview of peroxisomal disorders of metabolism and in addition discuss the toxicity of the intermediates of peroxisomal metabolism as they accumulate in the different peroxisomal deficiencies.
Wanders
R J A
RJ
University of Amsterdam, Academic Medical Center, Department of Clinical Chemistry, Laboratory of Genetic Metabolic Diseases, and Department of Pediatrics, Emma Children's Hospital, Laboratory of Genetic Metabolic Diseases, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
Ferdinandusse
S
S
Brites
P
P
Kemp
S
S
D000818:Animals; D004195:Disease Models, Animal; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D052439:Lipid Metabolism Disorders / Q000378:metabolism*; D015227:Lipid Peroxidation; D053858:Metabolic Networks and Pathways; D020675:Peroxisomes / Q000378:metabolism*
D005227:Fatty Acids