scholarly journals Congenital bile acid synthesis defect type 2

2020 ◽  
Author(s):  
Keyword(s):  
Bile Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Defect Type

scholarly journals Bile Acid Malabsorption in Chronic Diarrhea: Pathophysiology and Treatment

2013 ◽  
Vol 27 (11) ◽  
pp. 653-659 ◽  
Author(s):  
Alan Barkun ◽  
Jonathan Love ◽  
Michael Gould ◽  
Henryk Pluta ◽  
A Hillary Steinhart
Keyword(s):  
Bile Acid ◽  
High Performance ◽  
Chronic Diarrhea ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Bile Acid Malabsorption ◽  
Ileal Dysfunction ◽  
Type 3

BACKGROUND: Bile acid malabsorption (BAM) is a common but frequently under-recognized cause of chronic diarrhea, with an estimated prevalence of 4% to 5%.METHODS: The published literature for the period 1965 to 2012 was examined for articles regarding the pathophysiology and treatment of BAM to provide an overview of the management of BAM in gastroenterology practice.RESULTS: BAM is classified as type 1 (secondary to ileal dysfunction), type 2 (idiopathic) or type 3 (secondary to gastrointestinal disorders not associated with ileal dysfunction). The estimated prevalence of BAM is >90% in patients with resected Crohn disease (CD) and 11% to 52% of unresected CD patients (type 1); 33% in diarrhea-predominant irritable bowel syndrome (type 2); and is a frequent finding postcholecystectomy or postvagotomy (type 3). Investigations include BAM fecal bile acid assay, 23-seleno-25-homo-tauro-cholic acid (SeHCAT) testing and high-performance liquid chromatography of serum 7-α-OH-4-cholesten-3-one (C4), to determine the level of bile acid synthesis. A less time-consuming and expensive alternative in practice is an empirical trial of the bile acid sequestering agent cholestyramine. An estimated 70% to 96% of chronic diarrhea patients with BAM respond to short-course cholestyramine. Adverse effects include constipation, nausea, borborygmi, flatulence, bloating and abdominal pain. Other bile acid sequestering agents, such as colestipol and colesevelam, are currently being investigated for the treatment of BAM-associated diarrhea.CONCLUSIONS: BAM is a common cause of chronic diarrhea presenting in gastroenterology practice. In accordance with current guidelines, an empirical trial of a bile acid sequestering agent is warranted as part of the clinical workup to rule out BAM.

scholarly journals Curtailing FGF19’s mitogenicity by suppressing its receptor dimerization ability

2020 ◽  
Vol 117 (46) ◽  
pp. 29025-29034
Author(s):  
Jianlou Niu ◽  
Jing Zhao ◽  
Jiamin Wu ◽  
Guanting Qiao ◽  
Junlian Gu ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Cholestatic Liver Disease ◽  
Insulin Sensitizer ◽  
Diet Induced Obesity ◽  
Major Interest ◽  
Clinical Potential

As a physiological regulator of bile acid homeostasis, FGF19 is also a potent insulin sensitizer capable of normalizing plasma glucose concentration, improving lipid profile, ameliorating fatty liver disease, and causing weight loss in both diabetic and diet-induced obesity mice. There is therefore a major interest in developing FGF19 as a therapeutic agent for treating type 2 diabetes and cholestatic liver disease. However, the known tumorigenic risk associated with prolonged FGF19 administration is a major hurdle in realizing its clinical potential. Here, we show that nonmitogenic FGF19 variants that retain the full beneficial glucose-lowering and bile acid regulatory activities of WT FGF19 (FGF19WT) can be engineered by diminishing FGF19’s ability to induce dimerization of its cognate FGF receptors (FGFR). As proof of principle, we generated three such variants, each with a partial defect in binding affinity to FGFR (FGF19ΔFGFR) and its coreceptors, i.e., βklotho (FGF19ΔKLB) or heparan sulfate (FGF19ΔHBS). Pharmacological assays in WT anddb/dbmice confirmed that these variants incur a dramatic loss in mitogenic activity, yet are indistinguishable from FGF19WTin eliciting glycemic control and regulating bile acid synthesis. This approach provides a robust framework for the development of safer and more efficacious FGF19 analogs.

scholarly journals Endogenous Estrogens Lower Plasma PCSK9 and LDL Cholesterol But Not Lp(a) or Bile Acid Synthesis in Women

2012 ◽  
Vol 32 (3) ◽  
pp. 810-814 ◽  
Author(s):  
Lena Persson ◽  
Peter Henriksson ◽  
Eli Westerlund ◽  
Outi Hovatta ◽  
Bo Angelin ◽  
...  
Keyword(s):  
Bile Acid ◽  
Ldl Cholesterol ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Lower Plasma
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