Ferdinandusse Sacha, Houten Sander M
Laboratory Genetic Metabolic Diseases, Department of Clinical Chemistry, F0-224 Academic Medical Center at the University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands.
Biochim Biophys Acta. 2006 Dec;1763(12):1427-40. doi: 10.1016/j.bbamcr.2006.09.001. Epub 2006 Sep 14.
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 C24-bile acids from C27-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.
过氧化物酶体在胆汁酸的生物合成中发挥着重要作用，因为从C27胆汁酸中间体形成成熟的C24胆汁酸需要一个过氧化物酶体β-氧化步骤。此外，新合成的胆汁酸在过氧化物酶体内进行共轭反应。在这篇综述中，我们描述了目前关于健康和疾病状态下胆汁酸生物合成中过氧化物酶体功能各个方面的知识现状。已经鉴定出参与胆汁酸合成的过氧化物酶体酶，并讨论了其中一种酶缺乏的代谢和病理后果，包括核受体在其中的潜在作用。
