Metabolic and molecular basis of peroxisomal disorders: a review.
The group of peroxisomal disorders now includes 17 different disorders with Zellweger syndrome as prototype. Thanks to the explosion of new information about the functions and biogenesis of peroxisomes, the metabolic and molecular basis of most of the peroxisomal disorders has been resolved. A review of peroxisomal disorders is provided in this paper.
Wanders
Ronald J A
RJ
University of Amsterdam, Academic Medical Centre, Department of Clinical Chemistry, Emma Children's Hospital, Laboratory of Genetic Metabolic Diseases, Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
D000074183:ATPases Associated with Diverse Cellular Activities; D005227:Fatty Acids / Q000378:metabolism; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism; D008954:Models, Biological; D010084:Oxidation-Reduction; D018901:Peroxisomal Disorders / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism*; D020675:Peroxisomes / Q000378:metabolism
D005227:Fatty Acids; D008565:Membrane Proteins; D000074183:ATPases Associated with Diverse Cellular Activities; C111808:PEX1 protein, human