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During the last 80 years there have been extraordinary advances in our knowledge of the chemistry and biology of bile acids. We… Expand Biliary bile salt composition of 677 vertebrate species (103 fish, 130 reptiles, 271 birds, 173 mammals) was determined. Bile… Expand The formation of bile acids/bile alcohols is of major importance for the maintenance of cholesterol homeostasis. Besides their… Expand Abstract.Bile acids and bile alcohols in the form of their conjugates are amphipathic end products of cholesterol metabolism with… Expand In this report, we describe the first known patient with a deficiency of sterol carrier protein X (SCPx), a peroxisomal enzyme… Expand This review summarizes certain biochemical aspects of bile acid biosynthesis. It is pointed out that our knowledge of the… Expand The pregnane X receptor (PXR) regulates the metabolism and elimination of bile salts, steroids, and xenobiotics. The sequence of… Expand Duodenal bile, urine, plasma, and feces from a child with hepatic 3 beta-hydroxy-delta 5-C27-steroid dehydrogenase deficiency… Expand Bile acid production in cerebrotendinous xanthomatosis (CTX) is subnormal, yet the activity of cholesterol 7alpha-hydroxylase… Expand When brain tissue is homogenized in media iso-osmotic to plasma, the club-like presynaptic nerve endings resist disruption and… Expand