Results 181 to 190 of about 16,041 (210)

Metabolism of an oxysterol, 7‐ketocholesterol, by sterol 27‐hydroxylase in hepG2 cells

Lipids, 2001
Abstract7‐Ketocholesterol (7K) is a quantitatively important oxysterol in both atherosclerotic lesions and macrophage foam cells. We reported recently that radiolabeled 7K delivered to rodents in a modified lipoprotein or chylomicron remnantlike emulsion, both cleared predominantly by the liver, was rapidly excreted into the intestine as water‐soluble ...
M A, Lyons, A J, Brown
openaire   +2 more sources

Mutation in the Sterol 27‐Hydroxylase Gene Associated with Fatal Cholestasis in Infancy

Journal of Pediatric Gastroenterology and Nutrition, 2005
Inborn errors of bile acid synthesis are rare but potentially treatable causes of neonatal cholestasis. We here present a cholestatic infant with an ongoing cytomegalovirus infection who despite intensive treatment died of severe liver disease at 4 months of age.The urinary steroids were investigated by electrospray mass spectrometry and gas ...
Sara, von Bahr, Ingemar, Björkhem, Ferdinand, Van't Hooft, Gunvor, Alvelius, Antal, Nemeth, Jan, Sjövall, Björn, Fischler   +6 more
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Studies on sterol 27-hydroxylase (CYP27A1)

2004
Human sterol 27-hydroxylase (CYP27A1) has been studied in the present thesis with focus on substrate specificity and possible role of the enzyme for prevention of accumulation of cholesterol and cholestanol in tissues. Attempts have been made to evaluate a metabolic pathway to bile acids in human primary hepatocytes that starts with a 27-hydroxylation ...
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Functional analysis of the promoter of human sterol 27-hydroxylase gene in HepG2 cells

Gene, 2002
Human sterol 27-hydroxylase catalyses the first step in the alternative pathway of bile acids biosynthesis in hepatocytes. However the gene encoding this enzyme (CYP27 gene) is expressed in every tissue and some evidence suggests that this enzyme plays a role in cholesterol homeostasis.
GARUTI R, CROCE MA, PICCININI L, TIOZZO R, BERTOLINI, STEFANO, CALANDRA S.   +5 more
openaire   +3 more sources

Paradoxical enhancement of hepatic metabolism of 7-ketocholesterol in sterol 27-hydroxylase-deficient mice

Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, 2002
7-Ketocholesterol (7KC) is a major oxysterol found in atherosclerotic plaque and is believed to be derived both endogenously and exogenously (from the diet). Previously, we have demonstrated that subsequent to hepatic lipoprotein uptake, 7KC delivered in a model chylomicron remnant lipid emulsion is metabolised more rapidly and excreted into the ...
Malcolm A, Lyons, Nobuyo, Maeda, Andrew J, Brown   +2 more
openaire   +2 more sources

Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in a French family

Human Genetics, 1995
Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive lipid-storage disease caused by mutations in the sterol 27-hydroxylase gene (CYP27). So far several mutations causing CTX have been identified and characterized. A new mutation creating an insertion of cytosine at position 6 in the cDNA, which is expected to result in a frameshift and a ...
H, Segev, A, Reshef, V, Clavey, C, Delbart, G, Routier, E, Leitersdorf   +5 more
openaire   +2 more sources

Premature termination codon at the sterol 27-hydroxylase gene causes cerebrotendinous xanthomatosis in an Afrikaner family

Human Molecular Genetics, 1994
Cerebrotendinous xanthomatosis (CTX) is an autosomal recessive lipid-storage disease caused by mutations in the sterol 27-hydroxylase gene (CYP27). So far several mutations causing CTX have been identified and characterized. A new mutation creating an insertion of cytosine at position 6 in the cDNA, which is expected to result in a frameshift and a ...
V, Meiner, D A, Marais, A, Reshef, I, Björkhem, E, Leitersdorf   +4 more
openaire   +2 more sources

Competitive inhibition of hepatic sterol 27-hydroxylase by sitosterol: decreased activity in sitosterolemia.

Proceedings of the Association of American Physicians, 1998
We investigated the effect of sitosterol on hepatic sterol 27-hydroxylase activities in subjects with sitosterolemia, a recessive inherited disease associated with accelerated atherosclerosis and increased levels of sitosterol and other plant sterols and stanols in tissues.
L B, Nguyen, S, Shefer, G, Salen, S G, Tint, A K, Batta   +4 more
openaire   +1 more source

Transcriptional regulation of human sterol 27-hydroxylase gene in HepG2 cells

Atherosclerosis Supplements, 2001
R. Garuti, M.A. Croce, R. Tiozzo, S. Calandra   +3 more
openaire   +1 more source

Liver sterol 27 hydroxylase in hamster: Modulation by steroids and diets

Atherosclerosis, 2000
C. Lutton, M. Souidi, S. Dubrac, M. Parquet   +3 more
openaire   +1 more source