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Review
. 2009 Nov;50(11):2139-47.
doi: 10.1194/jlr.R900009-JLR200. Epub 2009 Apr 8.

Bile acids: the role of peroxisomes

Sacha Ferdinandusse  1 , Simone Denis, Phyllis L Faust, Ronald J A Wanders
Affiliations
Review

Bile acids: the role of peroxisomes

Sacha Ferdinandusse et al. J Lipid Res. 2009 Nov.

Abstract

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.

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Figures

Fig. 1.
Fig. 1.
Schematic representation of the peroxisomal steps involved in bile acid biosynthesis.
Fig. 2.
Fig. 2.
Bile acids measured in tissues of control subjects, patients with a peroxisome deficiency disorder (PDD), and patients with a D-bifunctional protein (DBP) deficiency. C24-bile acids are the sum of conjugated and unconjugated CA, CDCA, ursodeoxycholic acid, and deoxycholic acid. C27-bile acids are the sum of conjugated and unconjugated THCA, THCA:1, OH-THCA, OH-THCA:1, DHCA, DHCA:1, and C29DCA. Data represent the mean ± SD, all measurements were performed in duplicate. The number (n) of patients analyzed per tissue is indicated. *, P < 0.05; ** P < 0.005 (t-test).
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