Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.31 (beta-glucuronidase)
 7,680 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper describes the identification of a new bile alcohol possessing the 5 alpha-cholestane structure that was found in the urine of patients with cerebrotendinous xanthomatosis. The urine samples were extracted with reversed-phase resin, treated with beta-glucuronidase, and separated on silica gel and reversed-phase column chromatography. The new bile alcohol isolated was the second component of the urinary bile alcohols and was identified as (23S)-5 alpha-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol by means of gas-liquid chromatography/mass spectrometry and nuclear magnetic resonance spectroscopic studies.
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PMID:Identification of (23S)-5 alpha-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol in urine of patients with cerebrotendinous xanthomatosis. 180 58

Bile alcohol glucuronides present in human serum were isolated by ion exchange chromatography on piperidino-hydroxypropyl Sephadex LH-20. Following hydrolysis with beta-glucuronidase, the bile alcohols were analyzed by a combination of gas-liquid chromatography and mass spectrometry. Bile alcohols identified were 27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25-pentol, 27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25,26-hexol, 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25-pentol, 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,26-pentol, 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25,26-pentol, and 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25,26-hexol. The bile alcohol composition in serum was similar to that in urine but not to that in bile. The concentration of total bile alcohols in serum was 59.5 +/- 24.6 micrograms/L.
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PMID:Identification of bile alcohols in serum from healthy humans. 324 75

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 quantitated by gas-liquid chromatography-mass spectrometry. 5 beta-Cholestane-3 alpha,7 alpha,12 alpha,25-tetrol (334 micrograms/dl) was found to be the major bile alcohol, followed by 5 beta-cholestane-3 alpha,7 alpha,12 alpha,23R,25-pentol (65 micrograms/dl), and 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24(R and S),25-pentols (62.5 micrograms/dl and 64.5 micrograms/dl, respectively) in the plasma of these patients. When compared to biliary and urinary bile alcohol excretions, the plasma pattern resembled bile where 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol glucuronide predominated. In contrast, urinary bile alcohols were composed chiefly of 5 beta-cholestanepentol glucuronides with only small amounts of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol glucuronide. Treatment with chenodeoxycholic acid, which suppresses abnormal bile acid synthesis in these patients, reduced plasma bile alcohol concentrations dramatically. These results show that large quantities of bile alcohol glucuronides, particularly 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrolglucuronide, circulate in plasma of patients with cerebrotendinous xanthomatosis. The plasma bile alcohols closely resemble biliary bile alcohols which indicates their hepatic origin. The large quantities of polyhydroxylated bile alcohols in the urine may suggest their formation, at least in part, from 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25-tetrol by renal hydroxylating mechanisms.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Increased plasma bile alcohol glucuronides in patients with cerebrotendinous xanthomatosis: effect of chenodeoxycholic acid. 366 85

Bile alcohols present in urine from healthy adults were studied. Urine was extracted with octadecylsilane-bonded silica gel, and a neutral fraction and a glucuronide fraction were isolated by ion exchange chromatography on piperidinohydroxypropyl Sephadex LH-20. Following hydrolysis of the glucuronide fraction with beta-glucuronidase and purification by silica gel column chromatography, the bile alcohols were analyzed by a combined gas-liquid chromatography-mass spectrometry. By direct comparison with reference standards, the structures of four major bile alcohols were elucidated as follows: 27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25- pentol ; 5 beta-cholestane-3 alpha,7 alpha,12 alpha,24, 25- pentol ; 5 beta-cholestane-3 alpha,7 alpha,12 alpha,25,26- pentol ; and 5 beta-cholestane-3 alpha,7 alpha,12 alpha,26,27- pentol . The daily excretion of these four bile alcohols in urine was 0.5-1.0 mumoles. After purification by silica gel column chromatography, the neutral fraction was analyzed by a combined gas-liquid chromatography-mass spectrometry. The major bile alcohol excreted in urine as the unconjugated form was 27-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24,25- pentol . The daily excretion of the unconjugated C26 pentol (0.02-0.05 mumoles) was far lower than that of the conjugated C26 pentol (0.34-0.86 mumoles).
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PMID:Identification of bile alcohols in urine from healthy humans. 672 87

Using thin-layer chromatography, bile alcohol glucuronides were found with taurine- and glycine-conjugated bile acids in the bile of four patients with cerebrotendinous xanthomatosis. The concentration of the bile alcohol glucuronides was 1.7-5.2 times higher than that of the conjugated bile acids. Detectable amounts of unconjugated bile alcohols were not found in the bile of these patients. The bile alcohol glucuronides were isolated from the bile of one of the patients by means of preparative thin-layer chromatography. Treatment with beta-glucuronidase of the bile alcohol glucuronides liberated glucuronic acid and a mixture of bile alcohols. More than 90% of the liberated bile alcohols was 5 beta-cholestane-3 alpha, 7 alpha, 25-tetrol, and lesser amounts of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23-tetrol, 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 24-tetrol, 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 23, 25-pentol, and 5 beta-cholestane-3 alpha,-7 alpha, 12 alpha, 24 alpha, 25-pentol were also obtained. The bile alcohol glucuronides were not oxidized by the treatment with 3 alpha-hydroxysteroid dehydrogenase, indicating that the glucuronide moiety was at 3 alpha-hydroxyl position of the bile alcohols. Comparison of the mass spectra of the acetylated and methylated derivatives of the natural glucuronides and the synthetic 7 alpha, 12 alpha, 25-triacetoxy-5 beta-cholestan-3 alpha-O-(methyl 2,3,4-tri-O-acetyl-beta-D-glucopyranosyluronate) also indicated that the bile alcohol glucuronides consisted of mainly 5 beta - cholestane - 3 alpha, 7 alpha, 12 alpha, 25 - tetrol - glucuronide.
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PMID:Occurrence of bile alcohol glucuronides in bile of patients with cerebrotendinous xanthomatosis. 746 99