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.
机构:
Gdanski Uniwersytet Med, Katedra Biochem, Gdansk, Poland
Gdanski Uniwersytet Med, Zaklad Biochem, Gdansk, PolandGdanski Uniwersytet Med, Katedra Biochem, Gdansk, Poland
机构:
Univ London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, EnglandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England
Walters, Julian R. F.
Tasleem, Ali M.
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Univ London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, EnglandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England
Tasleem, Ali M.
Omer, Omer S.
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Univ London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, EnglandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England
Omer, Omer S.
Brydon, W. Gordon
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Western Gen Hosp, Dept Clin Chem, Edinburgh EH4 2XU, Midlothian, ScotlandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England
Brydon, W. Gordon
Dew, Tracy
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Kings Coll Hosp London, Dept Clin Biochem, London, EnglandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England
Dew, Tracy
Le Roux, Carel W.
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Univ London Imperial Coll Sci Technol & Med, Dept Metab Med, London W12 0HS, EnglandUniv London Imperial Coll Sci Technol & Med, Dept Gastroenterol, London W12 0HS, England