Bile acids: the role of peroxisomes

被引：88

作者：

Ferdinandusse, Sacha ^{[1
]}

Denis, Simone ^{[1
]}

Faust, Phyllis L. ^{[2
]}

Wanders, Ronald J. A. ^{[1
]}

机构：

[1] Univ Amsterdam, Acad Med Ctr, Lab Genet Metab Dis, NL-1105 AZ Amsterdam, Netherlands

[2] Columbia Univ, Dept Pathol & Cell Biol, New York, NY USA

来源：

JOURNAL OF LIPID RESEARCH | 2009年 / 50卷 / 11期

基金：

美国国家卫生研究院;

关键词：

dihydroxycholestanoic acid; trihydroxycholestanoic acid; peroxisome deficiency disorders; D-bifunctional protein deficiency; alpha-methylacyl-CoA racemase deficiency; METHYLACYL-COA RACEMASE; BIFUNCTIONAL PROTEIN-DEFICIENCY; RAT-LIVER; N-ACYLTRANSFERASE; ZELLWEGER-SYNDROME; TRIHYDROXYCHOLESTANOIC-ACID; MASS-SPECTROMETRY; FATTY-ACIDS; SUBCELLULAR-DISTRIBUTION; SUBSTRATE SPECIFICITIES;

D O I：

10.1194/jlr.R900009-JLR200

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.-Ferdinandusse, S., S. Denis, P. L. Faust, and R. J. A. Wanders. Bile acids: the role of peroxisomes. J. Lipid Res. 2009. 50: 2139-2147.

引用

页码：2139 / 2147

页数：9

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