Bile Acids: the Role of Peroxisomes

Overview

Journal J Lipid Res

Publisher Elsevier

Specialty Biochemistry

Date 2009 Apr 10

PMID 19357427

Citations 54

Authors

Sacha Ferdinandusse

Simone Denis

Phyllis L Faust

Ronald J A Wanders

Affiliations

Soon will be listed here.

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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