The Experts below are selected from a list of 102 Experts worldwide ranked by ideXlab platform
A Stiehl - One of the best experts on this subject based on the ideXlab platform.
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Transport of Chenodeoxycholic Acid and its 3-alpha- and 7-alpha-sulfates by isolated perfused rat liver.
Hepatology (Baltimore Md.), 1990Co-Authors: Ulrich Gartner, A Stiehl, R Raedsch, Tobias Goeser, Allan W. WolkoffAbstract:In patients with cholestasis, levels of sulfated bile Acids rise. Sulfate esters of Chenodeoxycholic Acid are the most abundant of these bile Acid sulfates. These compounds are taken up by the liver and excreted into bile, although their plasma clearance and biliary excretion are reduced compared with that of unsulfated bile Acids. It is not clear whether this is due to differences in intrinsic hepatic uptake or biliary excretion. In the present study, single-pass transport kinetics of Chenodeoxycholic Acid 3-alpha-sulfate, Chenodeoxycholic Acid 7-alpha-sulfate and unsulfated Chenodeoxycholic Acid were quantified in isolated perfused rat liver. Influx of the 7-alpha- and 3-alpha-sulfated derivatives was 57% and 20% that of Chenodeoxycholic Acid, respectively. These three compounds bound to albumin equally well, indicating that this was not a factor in their differential uptake. Although single-pass extraction of material taken up by the liver was identical. There was no difference in bile flow or biliary excretion of material taken up by the liver was identical. There was no difference in bile flow or biliary excretion rate, regardless of which bile Acid sulfate was tested. These results indicate that the low plasma elimination of sulfated bile Acids previously observed by others can be explained by low hepatic influx. The diminished transport into liver resulting from sulfation could lead to enhanced elimination of bile Acids by the kidney.
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acute effects of ursodeoxycholic and Chenodeoxycholic Acid on the small intestinal absorption of bile Acids
Gastroenterology, 1990Co-Authors: A Stiehl, R Raedsch, G RudolphAbstract:Abstract The effects of ursodeoxycholic Acid and Chenodeoxycholic Acid on the small-intestinal absorption of endogenous bile Acids were studied in patients with ileostomies who served as a model to investigate small-intestinal absorption in humans. In the control period, the eight patients excreted 327 ± 91 (mean ± standard error of the mean) μmol/8 h cholic Acid and 214 ± 38 μmol/8 h Chenodeoxycholic Acid by their ileal fluid. Following ursodeoxycholic Acid administration (500 mg), ileal excretion of cholic Acid increased to 517 ± 96 μmol/8 h, and that of Chenodeoxycholic Acid increased to 337 ± 42 μmol/8 h, indicating decreased absorption of these bile Acids. Following Chenodeoxycholic Acid administration (500 mg), no significant increase of cholic Acid excretion was observed, whereas Chenodeoxycholic Acid excretion increased as expected. It is concluded that following ursodeoxycholic Acid administration the absorption of common bile Acids from the small intestine decreases markedly. This effect of ursodeoxycholic Acid on intestinal absorption of common bile Acids probably is responsible for the decrease of their plasma concentrations, the reduction of their pool sizes, the increase of their fractional turnover rates, and most likely also contributes to the increased hepatic synthesis of cholic Acid.
A Warnet - One of the best experts on this subject based on the ideXlab platform.
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cerebrotendinous xanthornatosis treatments with simvastatin lovastatin and Chenodeoxycholic Acid in 3 siblings
Neurology, 1991Co-Authors: J Peynet, A Laurent, P De Liege, P Lecoz, P Gambert, A Legrand, J Mikol, A WarnetAbstract:We report 3 sisters treated for cerebrotendinous xanthomatosis. We treated one, with a severe neurologic form of the illness, with Chenodeoxycholic Acid, then lovastatin and simvastatin. These drugs had different efficacy and tolerance, but induced no clinical improvement. Her sisters, without neurologic symptoms, received Chenodeoxycholic Acid, which normalized the cholestanol level. Optimal treatment of this illness must begin before there is significant clinical symptomatology.
Kenji Ihara - One of the best experts on this subject based on the ideXlab platform.
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Two neonatal cholestasis patients with mutations in the SRD5B1 (AKR1D1) gene: diagnosis and bile Acid profiles during Chenodeoxycholic Acid treatment
Journal of Inherited Metabolic Disease, 2013Co-Authors: Yoshitaka Seki, Tatsuki Mizuochi, Akihiko Kimura, Tomoyuki Takahashi, Akira Ohtake, Shin-ichi Hayashi, Toshiya Morimura, Yasuharu Ohno, Takayuki Hoshina, Kenji IharaAbstract:Background and aims In two Japanese infants with neonatal cholestasis, 3-oxo-Δ^4-steroid 5β-reductase deficiency was diagnosed based on mutations of the SRD5B1 gene. Unusual bile Acids such as elevated 3-oxo-Δ^4 bile Acids were detected in their serum and urine by gas chromatography–mass spectrometry. We studied effects of oral Chenodeoxycholic Acid treatment. Patients and methods SRD5B1 gene analysis used peripheral lymphocyte genomic DNA. Diagnosis and treatment of these two patients were investigated retrospectively and prospectively investigated. Results With respect to SRD5B1 , one patient was heterozygous (R266Q, a novel mutation) while the other was a compound heterozygote (G223E/R261C). Chenodeoxycholic Acid treatment was effective in improving liver function and decreasing unusual bile Acids such as 7α-hydroxy- and 7α,12α-dihydroxy-3-oxo-4-cholen-24-oic Acids in serum and urine. Conclusion Primary bile Acid treatment using Chenodeoxycholic Acid was effective for these patients treated in early infancy before the late stage of chronic cholestatic liver dysfunction.
R Raedsch - One of the best experts on this subject based on the ideXlab platform.
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Transport of Chenodeoxycholic Acid and its 3-alpha- and 7-alpha-sulfates by isolated perfused rat liver.
Hepatology (Baltimore Md.), 1990Co-Authors: Ulrich Gartner, A Stiehl, R Raedsch, Tobias Goeser, Allan W. WolkoffAbstract:In patients with cholestasis, levels of sulfated bile Acids rise. Sulfate esters of Chenodeoxycholic Acid are the most abundant of these bile Acid sulfates. These compounds are taken up by the liver and excreted into bile, although their plasma clearance and biliary excretion are reduced compared with that of unsulfated bile Acids. It is not clear whether this is due to differences in intrinsic hepatic uptake or biliary excretion. In the present study, single-pass transport kinetics of Chenodeoxycholic Acid 3-alpha-sulfate, Chenodeoxycholic Acid 7-alpha-sulfate and unsulfated Chenodeoxycholic Acid were quantified in isolated perfused rat liver. Influx of the 7-alpha- and 3-alpha-sulfated derivatives was 57% and 20% that of Chenodeoxycholic Acid, respectively. These three compounds bound to albumin equally well, indicating that this was not a factor in their differential uptake. Although single-pass extraction of material taken up by the liver was identical. There was no difference in bile flow or biliary excretion of material taken up by the liver was identical. There was no difference in bile flow or biliary excretion rate, regardless of which bile Acid sulfate was tested. These results indicate that the low plasma elimination of sulfated bile Acids previously observed by others can be explained by low hepatic influx. The diminished transport into liver resulting from sulfation could lead to enhanced elimination of bile Acids by the kidney.
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acute effects of ursodeoxycholic and Chenodeoxycholic Acid on the small intestinal absorption of bile Acids
Gastroenterology, 1990Co-Authors: A Stiehl, R Raedsch, G RudolphAbstract:Abstract The effects of ursodeoxycholic Acid and Chenodeoxycholic Acid on the small-intestinal absorption of endogenous bile Acids were studied in patients with ileostomies who served as a model to investigate small-intestinal absorption in humans. In the control period, the eight patients excreted 327 ± 91 (mean ± standard error of the mean) μmol/8 h cholic Acid and 214 ± 38 μmol/8 h Chenodeoxycholic Acid by their ileal fluid. Following ursodeoxycholic Acid administration (500 mg), ileal excretion of cholic Acid increased to 517 ± 96 μmol/8 h, and that of Chenodeoxycholic Acid increased to 337 ± 42 μmol/8 h, indicating decreased absorption of these bile Acids. Following Chenodeoxycholic Acid administration (500 mg), no significant increase of cholic Acid excretion was observed, whereas Chenodeoxycholic Acid excretion increased as expected. It is concluded that following ursodeoxycholic Acid administration the absorption of common bile Acids from the small intestine decreases markedly. This effect of ursodeoxycholic Acid on intestinal absorption of common bile Acids probably is responsible for the decrease of their plasma concentrations, the reduction of their pool sizes, the increase of their fractional turnover rates, and most likely also contributes to the increased hepatic synthesis of cholic Acid.
Catherine Juste - One of the best experts on this subject based on the ideXlab platform.
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epimerization of Chenodeoxycholic Acid to ursodeoxycholic Acid by clostridium baratii isolated from human feces
Fems Microbiology Letters, 2004Co-Authors: Pascale Lepercq, Philippe Gerard, Fabienne Beguet, P Raibaud, Jeanpierre Grill, Purification Relano, Chantal Cayuela, Catherine JusteAbstract:Ursodeoxycholic Acid-producing bacteria are of clinical and industrial interest due to the multiple beneficial effects of this bile Acid on human health. This work reports the first isolation of 7-epimerizing bacteria from feces of a healthy volunteer, on the basis of their capacity to epimerize the primary bile Acid, Chenodeoxycholic Acid, to ursodeoxycholic Acid. Five isolates were found to be active starting from unconjugated Chenodeoxycholic Acid and its tauro-conjugated homologue, but none of these strains could epimerize the glyco-conjugated form. Biochemical testing and 16S ribosomal DNA sequencing converged to show that all five isolates were closely related to Clostridium baratii (99% sequence similarity), suggesting that this bacterial species could be responsible at least partially, for this bioconversion in the human gut.