Article

Bile acids: the role of peroxisomes.

Laboratory of Genetic Metabolic Diseases, Academic Medical Center at the University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
The Journal of Lipid Research (impact factor: 5.56). 05/2009; 50(11):2139-47. DOI:10.1194/jlr.R900009-JLR200 pp.2139-47
Source: PubMed

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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Keywords

abnormal bile acids
 
bile acid abnormalities
 
bile acid biosynthesis
 
bile acid measurements
 
bile acid synthesis
 
characteristic pattern
 
crucial role
 
de novo bile acid synthesis
 
distinct bile acid abnormalities
 
elucidation
 
pathophysiological consequences
 
patients
 
peroxisomal disorder
 
peroxisomal disorders
 
peroxisomes
 
precise role
 
toxicity