Peroxisornes and bile acid biosynthesis

被引:70
作者
Ferdinandusse, Sacha
Houten, Sander M.
机构
[1] Univ Amsterdam, Acad Med Ctr, Lab Genet Metab Dis, Dept Clin Chem, NL-1105 AZ Amsterdam, Netherlands
[2] Univ Amsterdam, Acad Med Ctr, Dept Pediat, NL-1105 AZ Amsterdam, Netherlands
来源
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH | 2006年 / 1763卷 / 12期
关键词
peroxisoinal beta-oxidation; nuclear receptors; C-27-bile acid intennediates; peroxisoine deficiency disorders;
D O I
10.1016/j.bbamcr.2006.09.001
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
Peroxisomes play an important role in the biosynthesis of bile acids because a peroxisomal beta-oxidation step is required for the formation of the mature C-24-bile acids from C-27-bile acid intermediates. In addition, de novo synthesized bile acids are conjugated within the peroxisome. In this review, we describe the current state of knowledge about all aspects of peroxisomal function in bile acid biosynthesis in health and disease. The peroxisomal enzymes involved in the synthesis of bile acids have been identified, and the metabolic and pathologic consequences of a deficiency of one of these enzymes are discussed, including the potential role of nuclear receptors therein. (c) 2006 Elsevier B.V. All rights reserved.
引用
收藏
页码:1427 / 1440
页数:14
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