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The enzymes, regulation, and genetics of bile acid synthesis.

来自 NCBI

阅读量：

565

作者：

DW Russell

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摘要：

The synthesis and excretion of bile acids comprise the major pathway of cholesterol catabolism in mammals. Synthesis provides a direct means of converting cholesterol, which is both hydrophobic and insoluble, into a water-soluble and readily excreted molecule, the bile acid. The biosynthetic steps that accomplish this transformation also confer detergent properties to the bile acid, which are exploited by the body to facilitate the secretion of cholesterol from the liver. This role in the elimination of cholesterol is counterbalanced by the ability of bile acids to solubilize dietary cholesterol and essential nutrients and to promote their delivery to the liver. The synthesis of a full complement of bile acids requires 17 enzymes. The expression of selected enzymes in the pathway is tightly regulated by nuclear hormone receptors and other transcription factors, which ensure a constant supply of bile acids in an ever changing metabolic environment. Inherited mutations that impair bile acid synthesis cause a spectrum of human disease; this ranges from liver failure in early childhood to progressive neuropathy in adults.

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关键词：

Animals Humans Liver Liver Diseases Enzymes Bile Acids and Salts Cholesterol Receptors, Cytoplasmic and Nuclear Homeostasis

DOI：

10.1146/annurev.biochem.72.121801.161712

被引量：

2632

年份：

2003

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来源期刊

Annual Review of Biochemistry
2003-11-28

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被引量：248

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