Metabolism of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 26-tetrol and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol into cholic acid in normal human subjects.
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منابع مشابه
Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid.
Oxidation of side chain of 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol was studied in a patient with cerebrotendinous xanthomatosis (CTX) and in control subjects, using various subcellular fractions of liver homogenate and a method based on isotope dilution-mass spectrometry. In the control, 5 beta-cholestane-3 alpha,7 alpha,12 alpha-triol was converted into 5 beta-cholestane-3 alpha,7 alp...
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Bile salts of ten species of frogs of the family Ranidae were examined by means of thin-layer chromatography, gas-liquid chromatography, and gas-liquid chromatography-mass spectrometry. Their major bile alcohols were ranol, cyprinol, or bufol. Four species of frogs contained lesser amounts of higher bile acids, and in two species C24 bile acids were present. A higher bile acid that has not been...
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Bile from Alligator mississippiensis was found to contain a mixture of more than twenty bile acids, bile alcohols, and neutral sterols. Bile acids and bile alcohols were purified by reversed-phase high performance liquid chromatography and thin-layer chromatography. Concentrations were measured by gas-liquid chromatography on 1% HiEFF-8BP and identifications were made by mass spectrometry. The ...
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Large quantities of C27 bile alcohols hydroxylated at C-25 are excreted in the bile and urine of patients with cerebrotendinous xanthomatosis, a lipid storage disease that results from defective bile acid synthesis. The presence of both biliary and urinary bile alcohols reflects impaired bile acid synthesis. After treatment of samples with beta-glucuronidase, plasma bile alcohols were quantitat...
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Bile formation and its canalicular secretion are essential functions of the mammalian liver. The sister-of-p-glycoprotein (spgp) gene was shown to encode the canalicular bile salt export protein, and mutations in spgp gene were identified as the cause of progressive familial intrahepatic cholestasis type 2. However, target inactivation of spgp gene in mice results in nonprogressive but persiste...
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ژورنال
عنوان ژورنال: Journal of Lipid Research
سال: 1979
ISSN: 0022-2275
DOI: 10.1016/s0022-2275(20)40603-0