Evidence for a role of the FXR-driven intestinal regulatory factor FGF-19 upon cholestyramine and chenodeoxycholic acid treatment and during the diurnal regulation of bile acid synthesis in humans

2007 ◽  
pp. 92-96
Author(s):  
M. Rudling ◽  
T. Lundasen ◽  
B. Angelin ◽  
C. Gälman
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Acid Treatment ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Regulatory Factor ◽  
Diurnal Regulation

scholarly journals Bile Acid Synthesis Disorders in Japan: Long-Term Outcome and Chenodeoxycholic Acid Treatment

2021 ◽  
Author(s):  
Akihiko Kimura ◽  
Tatsuki Mizuochi ◽  
Hajime Takei ◽  
Akira Ohtake ◽  
Jun Mori ◽  
...  
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Acid Treatment ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Recombinant 2-enoyl-CoA hydratase derived from rat peroxisomal multifunctional enzyme 2: role of the hydratase reaction in bile acid synthesis

1997 ◽  
Vol 328 (2) ◽  
pp. 377-382 ◽  
Author(s):  
Yong-Mei QIN ◽  
M. Antti HAAPALAINEN ◽  
Demara CONRY ◽  
A. Dean CUEBAS ◽  
J. Kalervo HILTUNEN ◽  
...  
Keyword(s):  
Bile Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Hydration Reaction ◽  
Multifunctional Enzymes ◽  
Hydratase Activity ◽  
Hydroxyacyl Coa Dehydrogenase ◽  
Liver Peroxisomes ◽  
Enoyl Coa Hydratase

Rat liver peroxisomes contain two multifunctional enzymes: (1) perMFE-1 [2-enoyl-CoA hydratase 1/Δ3,Δ2-enoyl-CoA isomerase/(S)-3-hydroxyacyl-CoA dehydrogenase] and (2) perMFE-2 [2-enoyl-CoA hydratase 2/(R)-3-hydroxyacyl-CoA dehydrogenase]. To investigate the role of the hydratase activity of perMFE-2 in β-oxidation, a truncated version of perMFE-2 was expressed in Escherichia coli as a recombinant protein. The protein catalyses the hydration of straight-chain (2E)-enoyl-CoAs to (3R)-hydroxyacyl-CoAs, but it is devoid of hydratase 1 [(2E)-enoyl-CoA to (3S)-hydroxyacyl-CoA] and (3R)-hydroxyacyl-CoA dehydrogenase activities. The purified enzyme (46 kDa hydratase 2) can be stored as an active enzyme for at least half a year. The recombinant enzyme hydrates (24E)-3α,7α,12α-trihydroxy- 5β-cholest-24-enoyl-CoA to (24R,25R)-3α,7α,12α,24-tetrahydroxy-5β-cholestanoyl-CoA, which has previously been characterized as a physiological intermediate in bile acid synthesis. The stereochemistry of the products indicates that the hydration reaction catalysed by the enzyme proceeds via a syn mechanism. A monofunctional 2-enoyl-CoA hydratase 2 has not been observed as a wild-type protein. The recombinant 46 kDa hydratase 2 described here survives in a purified form under storage, thus being the first protein of this type amenable to application as a tool in metabolic studies.

Role of the nuclear orphan receptor Rev-erb??; in the regulation of bile acid synthesis

2006 ◽  
Vol 18 (1) ◽  
pp. A44
Author(s):  
T Claudel ◽  
H Duez ◽  
J van der Veen ◽  
C Fontaine ◽  
R Havinga ◽  
...  
Keyword(s):  
Bile Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Orphan Receptor ◽  
Nuclear Orphan Receptor

Role of AMACR (α-methylacyl-CoA racemase) and MFE-1 (peroxisomal multifunctional enzyme-1) in bile acid synthesis in mice

2014 ◽  
Vol 461 (1) ◽  
pp. 125-135 ◽  
Author(s):  
Kaija J. Autio ◽  
Werner Schmitz ◽  
Remya R. Nair ◽  
Eija M. Selkälä ◽  
Raija T. Sormunen ◽  
...  
Keyword(s):  
Bile Acid ◽  
Mouse Models ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Wild Type ◽  
Multifunctional Enzyme

Bile acid analysis of wild-type, Mfe-1−/−, Amacr−/− and Amacr−/−Mfe-1−/− mouse models shows that peroxisomal multifunctional enzyme 1 can participate in bile acid synthesis in both AMACR-dependent and AMACR-independent pathways.

scholarly journals Regulation of bile-acid synthesis. Role of sterol carrier protein2 in the biosynthesis of 7α-hydroxycholesterol

1985 ◽  
Vol 230 (1) ◽  
pp. 19-24 ◽  
Author(s):  
H Seltman ◽  
W Diven ◽  
M Rizk ◽  
B J Noland ◽  
R Chanderbhan ◽  
...  
Keyword(s):  
Bile Acid ◽  
Rat Liver ◽  
Liver Microsomes ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Gas Chromatography Mass Spectrometry ◽  
Rat Liver Microsomes ◽  
Rate Limiting Step ◽  
Rate Limiting

Sterol carrier protein2 (SCP2) is known to stimulate utilization of cholesterol in enzymic reactions in which cholesterol is the substrate. Substantial recent experimental evidence indicates that SCP2: activates enzymic conversion of intermediates between lanosterol and cholesterol; stimulates the microsomal conversion of cholesterol into cholesterol ester in rat liver; and enhances mitochondrial utilization of cholesterol for pregnenolone formation in the adrenals. The conversion of cholesterol into 7 α-hydroxycholesterol is the rate-limiting step in bile-acid synthesis. We therefore investigated the effect of SCP2 on this physiologically critical reaction by using a gas-chromatography-mass-spectrometry procedure that measures the mass of 7 α-hydroxycholesterol formed. The results show that SCP2 enhances 7 α-hydroxycholesterol formation by rat liver microsomes (microsomal fractions), utilizing either endogenous membrane cholesterol, cholesterol supplied exogenously in serum or in the form of cholesterol/phospholipid liposomes. Microsomes immunotitrated with anti-SCP2 antibody exhibited considerably less capacity to synthesize 7 α-hydroxycholesterol, which was restored to control levels on addition of purified SCP2. These data are consistent with the suggestion that SCP2 may be of physiological significance in the overall metabolism of cholesterol.

scholarly journals Complex feedback regulation of bile acid synthesis in the hamster: The role of newly synthesized cholesterol

Hepatology ◽  
1999 ◽  
Vol 30 (1) ◽  
pp. 230-237 ◽  
Author(s):  
Jürgen Scheibner ◽  
Michael Fuchs ◽  
Erwin Hörmann ◽  
Eduard F. Stange
Keyword(s):  
Bile Acid ◽  
Feedback Regulation ◽  
Acid Synthesis ◽  
Bile Acid Synthesis

Bile acid synthesis disorders in Japan: long-term outcome and chenodeoxycholic acid treatment

2020 ◽  
Author(s):  
Akihiko Kimura ◽  
Tatsuki Mizuochi ◽  
Hajime Takei ◽  
Akira Ohtake ◽  
Jun Mori ◽  
...  
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Low Dose ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Reductase Deficiency ◽  
Clinical Records

Abstract Background: We encountered 7 Japanese patients with bile acid synthesis disorders (BASD) over 21 years between 1996 and 2017. Diagnoses were made by bile acid and genetic analyses. We gave low-dose, long-term chenodeoxycholic acid (CDCA) treatment to 5 of the patients, who had 3β-hydroxy-Δ 5 -C 27 -steroid dehydrogenase/isomerase (3β-HSD) deficiency (n=3) or Δ 4 -3-oxosteroid 5β-reductase (5β-reductase) deficiency (n=2). Another patient with the latter diagnosis whose bile acid analyses had mitigating features was maintained on ursodeoxycholic acid according to parental preferences and now remains healthy after discontinuation of treatment. A patient with oxysterol 7α-hydroxylase deficiency developed liver failure and fully recovered after successful liver transplantation. We used clinical records to clarify long-term outcome and value of CDCA in the other patients. Efficacy of CDCA treatment was evaluated in the 5 patients given a low dose (5 to 10 mg/kg/day) for a long term. Results: Medians with ranges of current patient ages and duration of CDCA treatment are10 years (8 to 43) and 10 years (8 to 21), respectively. All 7 patients, who had homozygous or compound heterozygous mutations in the HSD3B7 , SRD5B1 , or CYP7B1 gene, are currently in good health without liver dysfunction. In the 5 patients with CDCA treatment, hepatic function gradually improved following initiation. No adverse effects were noted. Conclusions: We concluded that low-dose CDCA treatment is effective in 3β-HSD deficiency and 5β-reductase deficiency, as cholic acid has been in other countries. BASD carry a good prognosis following early diagnosis and initiation of long-term, low-dose CDCA treatment.

Th-W52:7 Influence of age on bile acid synthesis: Role of hepatic expression of nuclear receptors

2006 ◽  
Vol 7 (3) ◽  
pp. 472
Author(s):  
C. Gabbi ◽  
M. Bertolotti ◽  
C. Anzivino ◽  
D. Macchioni ◽  
M. Crestani ◽  
...  
Keyword(s):  
Bile Acid ◽  
Nuclear Receptors ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Hepatic Expression
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