Peroxisomes and bile acid biosynthesis.
Peroxisomes play an important role in the biosynthesis of bile acids because a peroxisomal beta-oxidation step is required for the formation of the mature C24-bile acids from C27-bile acid intermediates. In addition, de novo synthesized bile acids are conjugated within the peroxisome. In this review, we describe the current state of knowledge about all aspects of peroxisomal function in bile acid biosynthesis in health and disease. The peroxisomal enzymes involved in the synthesis of bile acids have been identified, and the metabolic and pathologic consequences of a deficiency of one of these enzymes are discussed, including the potential role of nuclear receptors therein.
Ferdinandusse
Sacha
S
Laboratory Genetic Metabolic Diseases, Department of Clinical Chemistry, F0-224 Academic Medical Center at the University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands. S.Ferdinandusse@amc.uva.nl
Houten
Sander M
SM
D000818:Animals; D001647:Bile Acids and Salts / Q000096:biosynthesis*; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D010084:Oxidation-Reduction; D018901:Peroxisomal Disorders / Q000235:genetics / Q000378:metabolism*; D020675:Peroxisomes / Q000235:genetics / Q000502:physiology*
D001647:Bile Acids and Salts