Bile acid synthesis
Identification of a new inborn error in bile acid synthesis: mutation of the oxysterol 7alpha hydroxylase gene causes severe neonatal liver disease
... in bile acid synthesis involv- ing a deficiency in 7 a -hydroxylation due to a mutation in the gene for the microsomal oxysterol 7 a -hydroxylase en- zyme, active in the acidic pathway for ...
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Role of primary and secondary bile acids as feedback inhibitors of bile acid synthesis in the rat in vivo
... secondary bile acids on the rates of synthesis of all major bile acids was studied in the live rat with an extracorporal bile ...duct. Bile acid synthesis was determined ...
9
Bile Acid Synthesis in the Isolated, Perfused Rabbit Liver
... the bile contained ...the bile acids had been interrupted sufficiently to release the feedback inhibition of the rate-controlling enzyme of bile acid ...into bile acids at a more rapid ...
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Feedback regulation of bile acid synthesis in the rat Differing effects of taurocholate and tauroursocholate
... during bile- acid ...increase bile-acid synthesis and cholesterol 7 alpha-hydroxylase ...hepatic bile-acid flux), bile-acid synthesis decreased 82% ...
9
Impaired negative feedback suppression of bile acid synthesis in mice lacking βKlotho
... The synthesis and excretion of bile acids were found to be dramatically elevated in these mutants, and the expres- sion of 2 key bile acid synthase genes, cholesterol 7α-hydroxylase (Cyp7a1) ...
8
Ketoconazole blocks bile acid synthesis in hepatocyte monolayer cultures and in vivo in rat by inhibiting cholesterol 7 alpha hydroxylase
... In bile-diverted rats a single dose of ketoconazole (50 mg/kg) dramatically impaired bile flow and biliary bile acid output (92% ...for bile acid ...inhibit bile ...
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Regulation of bile acid synthesis in man Presence of a diurnal rhythm
... of bile acid synthesis in three human subjects given ...to bile acid, the 14C is released as propionic acid randomly labeled in the 1 and 3 ...propionic acid is then ...
8
Bile acid synthesis in man In vivo activity of the 25 hydroxylation pathway
... [14C]Acetone production averaged 4.9% of total release of 14C from [14C]26-cholesterol, estimated by 14CO2 output. The method was validated by showing that [14C]acetone production from [14C]isopropanol averaged 86.9% of ...
5
Cholic acid mediates negative feedback regulation of bile acid synthesis in mice
... ileal bile acid binding protein (IBABP), CAAGGCTACCGTGAAGATGGA and CCCACGACCTCC- GAAGTCT ; cyclophilin, TGGAGAGCACCAAGACAGACA and TGCCGGAGTCGACAATGAT ; and GAPDH, TGTGTC- CGTCGTGGATCTGA and ...
11
THE RELATION OF PLASMA AND BILIARY CHOLESTEROL TO BILE ACID SYNTHESIS IN MAN
... A similar elevation in the specific activity of biliary cholesterol as compared with plasma cholesterol was reported by Gould and coworkers who administered labeled acetate to dogs with [r] ...
6
Elevated Cholesterol and Bile Acid Synthesis in a Young Patient with Homozygous Familial Hypercholesterolemia
... and bile acid excretion was ...and bile acids, ...and bile acid outputs in the normal child were ...in synthesis of cholesterol and bile acids. Although synthesis ...
6
The influence of biological sex and sex hormones on bile acid synthesis and cholesterol homeostasis
... BA synthesis, with ab- normalities in their expression or function leading to liver, digestive, and systemic pathologies secondary to el- evated cholesterol [1, ...cholic acid (CA) and chenodeoxycholic ...
12
Dietary fish oil induced changes in intrahepatic cholesterol transport and bile acid synthesis in rats
... Hepatic cholesterol metabolism was studied in rats fed purified diets supplemented (9% wt/wt) with either fish oil (FO) (n-3 fatty acids) or corn oil (CO) (n-6 fatty acids) for 4 wk. Rats were equipped with permanent ...
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The bile acid synthetic gene 3β hydroxy Δ5 C27 steroid oxidoreductase is mutated in progressive intrahepatic cholestasis
... of bile acid ...in bile acid pool ...of bile acids and provide molecular tools for the diagnosis of a third type of neonatal progressive intrahepatic cholestasis associated with ...
11
Cholesterol kinetics in subjects with bile fistula Positive relationship between size of the bile acid precursor pool and bile acid synthetic rate
... studying bile fistula subjects, using several types of isotopic preparations, simultaneous labeling of separate cholesterol pools and sampling all components of blood and bile at frequent intervals, we ...
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Disorders of bile acid metabolism in cholesterol gallstone disease
... chenodeoxycholic acid (CDCA) in 23 female gallstone patients classified according to their gallbladder function and in 15 healthy female ...hepatic bile acid synthesis, but, depending on ...
11
Heterogeneous expression of cholesterol 7 alpha hydroxylase and sterol 27 hydroxylase genes in the rat liver lobulus
... We investigated the lobular localization and molecular level of expression of cholesterol 7 alpha-hydroxylase and sterol 27-hydroxylase, two key enzymes in bile acid synthesis, in isolated periportal ...
10
Hepatic nuclear corepressor 1 regulates cholesterol absorption through a TRβ1 governed pathway
... of bile acid synthesis enzymes, we used a model recently developed and used for ChIP sequencing by our laboratory ...these bile acid synthesis and transport genes by recruitment ...
12
Risk factors associated with mortality in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and clinical implications
... amino acid profiles, and misdiagnosis (such as tyrosinemia type 1, or bile acid synthesis defect) may all have contributed to late re- ferral or delayed switch to LF/MCT ...
8
The intestinal microbiota regulates host cholesterol homeostasis
... cholesterol synthesis, lipoprotein uptake by the liver, bile acid synthesis, and intestinal absorption ...cholesterol synthesis such as HmgcoAr expression and lathosterol concentra- ...
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