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Journal of Lipid Research, Vol. 50, 2139-2147, November 2009
Copyright © 2009 by American Society for Biochemistry and Molecular Biology

Thematic Review

Bile acids: the role of peroxisomes

Sacha Ferdinandusse^1,*, Simone Denis^*, Phyllis L. Faust and Ronald J. A. Wanders^*

^* Laboratory of Genetic Metabolic Diseases, Academic Medical Center at the University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Department of Pathology and Cell Biology, Columbia University, New York, NY

¹ To whom correspondence should be addressed. e-mail: S.Ferdinandusse{at}amc.uva.nl

It is well established that peroxisomes play a crucial role in de novo bile acid synthesis. Studies in patients with a peroxisomal disorder have been indispensable for the elucidation of the precise role of peroxisomes. Several peroxisomal disorders are associated with distinct bile acid abnormalities and each disorder has a characteristic pattern of abnormal bile acids that accumulate, which is often used for diagnostic purposes. The patients have also been important for determining the pathophysiological consequences of defects in bile acid biosynthesis. In this review, we will discuss all the peroxisomal steps involved in bile acid synthesis and the bile acid abnormalities in patients with peroxisomal disorders. We will show the results of bile acid measurements in several tissues from patients, including brain, and we will discuss the toxicity and the pathological effects of the abnormal bile acids.

Supplementary key words dihydroxycholestanoic acid • trihydroxy-cholestanoic acid • peroxisome deficiency disorders • D-bifunctional protein deficiency • -methylacyl-CoA racemase deficiency

Abbreviations: AMACR, -methylacyl-CoA racemase; BACS, bile acyl-CoA synthetase; BAAT, bile acyl-CoA: amino acid N-acyltransferase; BCOX, branched-chain acyl-CoA oxidase; CA, cholic acid; CDCA, chenodeoxycholic acid; DBP, D-bifunctional protein; DCA, deoxycholic acid; DHCA, dihydroxycholestanoic acid; FXR, farnesoid X receptor; SCPx, sterol carrier protein X; THCA, trihydroxycholestanoic acid; UDCA, ursodeoxycholic acid

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