期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
卷 1763, 期 12, 页码 1427-1440出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2006.09.001
关键词
peroxisoinal beta-oxidation; nuclear receptors; C-27-bile acid intennediates; peroxisoine deficiency disorders
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.
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