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A novel etiologic factor of highly elevated cholestanol levels: progressive familial intrahepatic cholestasis

Journal of Pediatric Endocrinology & Metabolism (JPEM), 2020
Background Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an uncommon cholestatic liver disease caused by mutations in the ATP binding cassette subfamily B member 4 (ABCB4) gene.
Aynur Küçükçongar Yavaş   +4 more
semanticscholar   +1 more source

Cholestanol Esters of Amino Acids

Bulletin of the Chemical Society of Japan, 1967
Abstract The cholestanol (5α-cholestan-3β-ol) esters of amino acids were synthesized with an interest for their possible biochemical activities. The cholestanol esters of glycine, l-alanine, β-alanine, l-leucine and l-aspartic acid were synthesized by a direct fusion of the two components under the passing of hydrogen chloride through ...
Y, Matsumoto, M, Shirai, M, Minagawa
openaire   +2 more sources

Prevention of Cholestanol-induced Cholelithiasis by Neomycin

Nature, 1965
RABBITS receiving 0.25–1 per cent dietary cholestanol (5α-cholestan-3β-ol) for 3–6 weeks develop gall-stones which have been shown to consist predominantly of calcium, and sodium glycoallodeoxycholate (3α-, 12α-dihydroxy-5α-cholanoyl glycine)1,2. Gall-stone formation in cholestanol-fed rabbits has been postulated to occur as follows: Cholestanol is ...
E H, Mosbach   +5 more
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Chromatographic separation of putative precursors of cholestanol

Steroids, 1988
This paper describes convenient syntheses for labeled and unlabeled cholest-5-en-3-one, cholest-4-en-3-one, epicholesterol, cholest-4-en-3 beta-ol, and cholest-4-en-3 alpha-ol. The thin-layer chromatography, high-performance liquid chromatography, and gas-liquid chromatography of these compounds and of cholestanol and epicholestanol are also described.
A K, Batta, G, Salen, S, Shefer
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Cholestanol-Induced Gallstones: Radiologic Observations

Radiology, 1967
Gallstones can be reliably induced in rabbits by the addition of 3-beta-cholestanol to an otherwise normal diet. This initial observation of Bevans and Mosbach (2) has been confirmed by others (3, 6, 8) and has been found to be a useful experimental model.
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Squalene and cholestanol in normal rabbit aorta

Atherosclerosis, 1970
Abstract Squalene and cholestanol were isolated from normal rabbit aortic tissue, identified and quantitatively determined. The concentration of squalene and cholestanol was 4.5 and 7.7 mg respectively, in 100 g of dry, delipidized tissue.
V, Stefanovich, G, Kajiyama
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Analysis of sterol extracts for cholestanol

Analytical Biochemistry, 1965
Abstract Cholestanol analyses of sterols have been carried out by removal of the unsaturated sterols by oxidation and extraction followed by gas chromatography of the saturated sterols. The method can detect 0.2% of cholestanol in sterol mixtures.
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The metabolism of cholestanol in primary biliary cirrhosis

Journal of Hepatology, 1996
The concentration of serum cholestanol, a 5 alpha-saturated derivative of cholesterol, is increased in primary biliary cirrhosis proportionally to impaired liver function for unknown reasons. The purpose of this study was to analyze serum cholestanol level and its biliary and fecal elimination, and relate the results to cholesterol absorption and ...
H, Gylling   +3 more
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Effects of cholestanol feeding on corneal dystrophy in mice

Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1991
A cholestanol-enriched diet administered for 8 months to BALB/c mice produced in 20% two kinds of corneal opacities resembling calcific band keratopathy and Schnyder's crystalline dystrophy in humans. The concentrations of cholestanol in serum, liver and cornea of the corneal opacity bearing mice were 30-40-times higher than those of normal mice.
K S, Kim   +5 more
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Cholestanol and plant sterols in pigeon skin

Lipids, 1973
AbstractCholestanol (4.6%) and plant sterols (0.2%) have been demonstrated, for the first time, in avian skin by argentation and gas liquid chromatography. In contrast to results of previous studies with rat and human skin, cholestanol represented a significant amount in the pigeon.
openaire   +2 more sources

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