The Enzymes, Regulation, and Genetics of Bile Acid Synthesis

Russell, David W.

doi:10.1146/annurev.biochem.72.121801.161712

Cited by 2,069Cholesterol and Lipid MetabolismDrug Transport and Resistance MechanismsSteroid Chemistry and Biochemistry

The Enzymes, Regulation, and Genetics of Bile Acid Synthesis

Russell, David W.·2003·Annual Review of Biochemistry

DOI 10.1146/annurev.biochem.72.121801.161712

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Abstract

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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Primary bile acids are synthesized in the liver from cholesterol: cholic acid and chenodeoxycholic acid in humans, and cholic acid and α/β-muricholic acid in mice.
“Primary bile acids are synthesized in the liver from cholesterol: cholic acid and chenodeoxycholic acid in humans, and cholic acid and α/β-muricholic acid in mice (Russell, 2003).”
From The Microbiota-Gut-Brain Axis (2019) · cites this paper as Russell, 2003

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Authors: Russell, D. W.
Affiliations: The University of Texas Southwestern Medical Center (US)
Corresponding author: Russell, David W.
ORCID: Russell, David W.
Venue: Annual Review of Biochemistry
Publisher: Annual Reviews
Published: 2003-06
Volume: 72
Issue: 1
Pages: 137-174
ISSN: 0066-4154 (ISSN-L)
1545-4509
DOI: 10.1146/annurev.biochem.72.121801.161712
OpenAlex: W2106515170
Cited by: 2,069
References: 6
Citations by year: 2026: 99 · 2025: 107 · 2024: 139 · 2023: 130 · 2022: 150 · 2021: 145 · 2020: 103 · 2019: 119 · 2018: 113 · 2017: 108 · 2016: 79 · 2015: 80 · 2014: 107 · 2013: 72 · 2012: 55
FWCI: 8.55
Type: journal article
Language: en
Open access: closed
Locations: Annual Review of Biochemistry (publishedVersion)
PubMed (publishedVersion)
Funders: National Institutes of Health (5r01hd038127-14)
National Heart, Lung, and Blood Institute (HL-20948, P01HL020948)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD38127)
Topics: Health Sciences › Medicine › Surgery › Cholesterol and Lipid Metabolism
Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Steroid Chemistry and Biochemistry
Keywords: bile acid synthesis, cholesterol catabolism, nuclear receptors, transcriptional regulation, bile acid excretion, cholesterol solubilization, liver failure
MeSH terms: Animals, Bile Acids and Salts, Cholesterol, Enzymes, Homeostasis, Humans, Liver, Liver Diseases, Receptors, Cytoplasmic and Nuclear

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The Enzymes, Regulation, and Genetics of Bile Acid Synthesis

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