Genes involved in initial steps of bile acid synthesis.
The mechanism and regulation of the degradation of cholesterol into bile acids has attracted increased interest, in particular after the recent discovery that nuclear receptors (farnesoid X receptor and liver X receptor) are involved in the regulation of bile acid synthesis. Recently, it has also been shown that the biosynthesis of bile acids is not exclusively restricted to the liver, and that degradation may start by a hydroxylation of cholesterol in the brain or in other extrahepatic organs. During the past 2 years the genes coding for three of the six enzymes catalysing the first steps in bile acid biosynthesis have been cloned and characterized. These genes and their gene products will be described here.
Division of Clinical Chemistry, Karolinska Institutet, Huddinge University Hospital, Huddinge, Sweden. Ingemar.Bjorkhem@chemlab.hs.sll.se
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Björkhem
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Eggertsen
D015096:3-Hydroxysteroid Dehydrogenases / Q000235:genetics / Q000378:metabolism; D001647:Bile Acids and Salts / Q000096:biosynthesis* / Q000235:genetics*; D003577:Cytochrome P-450 Enzyme System / Q000235:genetics / Q000378:metabolism; D006801:Humans
D001647:Bile Acids and Salts; D003577:Cytochrome P-450 Enzyme System; D015096:3-Hydroxysteroid Dehydrogenases