Significance of bile alcohol in urine of a patient with cholestasis: Identification of 5.BETA.-cholestane-3.ALPHA.,7.ALPHA.,12.ALPHA.,26,27-pentol (5.BETA.-cyprinol) and 5.BETA.-cholestane-3.ALPHA.,7.ALPHA.,12.ALPHA.,26-tetrol (27-deoxy-5.BETA.-cyprinol).
نویسندگان
چکیده
منابع مشابه
Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27(-/-) mice and CTX.
Cerebrotendinous xanthomatosis (CTX) is a rare, recessively inherited lipid storage disease characterized by a markedly reduced production of chenodeoxycholic acid and an increased formation of 25-hydroxylated bile alcohols and cholestanol. Patients with this disease are known to have mutations in the sterol 27-hydroxylase (Cyp27) gene. However, one study showed that mice with a disrupted Cyp27...
متن کاملSide chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis.
The accumulation of various 25-hydroxylated C(27)-bile alcohols in blood and their excretion in urine are characteristic features of cerebrotendinous xanthomatosis (CTX) a recessively inherited inborn error of bile acid synthesis caused by mutations in the mitochondrial sterol 27-hydroxylase (CYP27) gene. These bile alcohols may be intermediates in the alternative cholic acid side chain cleavag...
متن کاملAbsolute configuration of pentahydroxy bile alcohols excreted by patients with cerebrotendinous xanthomatosis: a circular dichroism study.
The absolute configurations of the C27 pentahydroxy bile alcohols present in bile and feces of two patients with cerebrotendinous xanthomatosis (CTX) were determined by circular dichroism (CD) spectroscopy. The CD spectra of 5beta-cholestane-3alpha,7alpha,12alpha,24alpha,25-pentol in the presence of Eu(fod)3 [tris(1,1,1,2,2,3,3-heptafluoro-7,7-dimethyloctane-4,6-dionato) europium (III)] exhibit...
متن کاملRegulation of 25- and 27-hydroxylation side chain cleavage pathways for cholic acid biosynthesis in humans, rabbits, and mice. Assay of enzyme activities by high-resolution gas chromatography;-mass spectrometry.
In classic cholic acid biosynthesis, a series of ring modifications of cholesterol precede side chain cleavage and yield 5beta-cholestane-3alpha, 7alpha, 12alpha-triol. Side chain reactions of the triol then proceed either by the mitochondrial 27-hydroxylation pathway or by the microsomal 25-hydroxylation pathway. We have developed specific and precise assay methods to measure the activities of...
متن کاملNuclear-magnetic-resonance and mass-spectral study of myxinol tetra-acetate.
Myxinol tetra-acetate was examined by both nuclear-magnetic-resonance and mass-spectral techniques. The results are compared with corresponding data for 5alpha-ranol tetra-acetate and 5alpha-cyprinol tetra-acetate. The structure deduced for myxinol tetra-acetate, 3beta,7alpha,16alpha,27-tetrahydroxy-5beta-cholestane, is in agreement with that simultaneously arrived at by Haslewood (1966).
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ژورنال
عنوان ژورنال: Chemical and Pharmaceutical Bulletin
سال: 1984
ISSN: 0009-2363,1347-5223
DOI: 10.1248/cpb.32.2874