Bile Acid Synthesis, Metabolism and Biological Functions
(2010b) A new inborn error of bile acid synthesis - bile acid-CoA ligase deficiency.
Bile Acid Synthesis and Utilization
(2003) Molecular genetics of 3-beta-hydroxy-D5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease.
Dietary fiber appears to deter the synthesis and absorption of secondary bile acids which are unhealthy bile metabolites that form in the intestinal tract.
The Synthesis of Cholesterol. - Cholesterol-And …
Cholesterol biosynthesis and hepatic uptake from blood. a Real-time quantitative PCR of genes involved in hepatic cholesterol synthesis and uptake, including SREBP-2, HMG-CoA reductase, LDLR and LRP1. b Western blot analysis and representative images of SREBP-2, HMG-CoA reductase, LDLR and LRP1. n = 6/group. a P †,†† means significance (P HFC high fat-cholesterol, HMG-CoA 3-hydroxy-3-methylglutaryl CoA, LDLR low-density lipoprotein receptor, LRP1 low-density lipoprotein receptor-related protein-1, SP stroke-prone, SREBP-2 sterol regulatory element-binding protein-2
These medications are typically effective in eliminating the symptoms of BAD. However, when the medications are prescribed at the dosage for the condition they have FDA approval, e.g. high cholesterol, constipation and other digestive symptoms may be experienced. If you have been prescribed one of these medications, it is important to work with your doctor to find a dosage that is right for you.
Bile acids are synthesized in ..
Gamma Glutamyltransferase: GGT – This enzyme is has its highest concentration in the kidneys and pancreas, but it is also found in the liver and other organs. The major proportion of GGT in the serum seems to come from the liver. Elevations of GGT in disease seem to stem from new synthesis rather than leakage, therefore the changes seen due to disease are not spectacular. Large elevations of GGT are more commonly associated with pancreatitis and bile duct obstruction.
The disorders of peroxisome biogenesis and peroxisomal β-oxidation that affect bile acid synthesis will be covered in the review by Ferdinandusse et al.
Cholesterol synthesis steps and regulation - SlideShare
Disorders of bile acid synthesis | SpringerLink
The explanation for this paradox may be that the liver synthesizes less cholesterol as dietary cholesterol increases.
Dysregulated Bile Acid Synthesis, Metabolism and …
(2007) Cholestatic liver disease in adults may be due to an inherited defect in bile acid biosynthesis.
The greatest proportion of cholesterol is used in bile acid synthesis
(2009) Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy.
Biosynthesis of Cholesterol | The Biochemistry …
The disorders that are discussed in this review are: 3β-hydroxysteroid-Δ5-C27-steroid dehydrogenase deficiency, Δ4-3-oxosteroid 5β-reductase deficiency, sterol 27-hydroxylase deficiency (cerberotendinous xanthomatosis, CTX), oxysterol 7α-hydroxylase deficiency (including one form of hereditary spastic paraparesis) and the amidation defects, bile acid-CoA: aminoacid N-acyltransferase (BAAT) deficiency and bile acid-CoA ligase deficiency.
Bile Acid Synthesis Disorders - Group Health Cooperative
The researchers theorize that oxidant stress within the liver leads to unfavorable changes in bile composition that promote precipitation of both cholesterol and bilirubin These findings corroborate earlier studies showing that antioxidant deficiencies can induce gallstone formation in animals.
The Synthesis of Cholesterol. - Cholesterol - You Can't …
A stroke-prone (SP: 20.8 % crude protein, 4.8 % crude lipid, 3.2 % crude fiber, 5.0 % crude ash, 8.0 % moisture, and 58.2 % carbohydrate) diet as a control diet and HFC diet (a mixture of 68 % SP diet, 25 % palm oil, 5 % cholesterol and 2 % cholic acid) were obtained from Funabashi Farm (Chiba, Japan). The components of each diet have been shown elsewhere .
The end products of cholesterol utilization are the bile acids
Biliary compounds such as BAs and bilirubin are cytotoxic when present in abnormally high concentrations. Both glucuronidation and sulfation become pivotal eliminating pathways in cholestasis. They transform hydrophobic, toxic substrates into more hydrophilic, less-toxic derivatives for biliary and urinary excretion, although glycine and taurine conjugation are primarily involved in the process [, ]. The most profound changes in the pathways of BA turnover in this dietary model were disturbed UGT-catalyzed glucuronidation, blunted SULT2A1-catalyzed sulfation, and impaired BSEP-mediated canalicular export to bile duct, especially observed at 8 and 14 weeks, when fibrosis progressed. All of these disorders, together with upregulations of BA synthetic enzymes, revealed hepatic accumulation of toxic biliary constituents, as evidenced by a direct analysis of BA profiles in liver (Jia X et al., manuscript in submission). Consequently, toxic biliary compound overload in liver may have contributed to the pathogenesis of liver damage in this model, especially severe fibrosis, as confirmed by the correlation analysis between specific BAs and parameters of liver injury (Jia X et al., manuscript in submission). HFC-diet feeding also induced MRP3-regulated basolateral excretion, an important alternative spillover route during BA overload, which was in agreement with the changes under a cholestatic condition . These changes allow BA elimination from liver to the blood, but are not sufficient for a complete detoxification of the liver cells.
Glossary | Linus Pauling Institute | Oregon State University
Unlike the control diet, the HFC diet deposited cholesterol greatly in rat livers, where 3-hydroxy-3-methylglutaryl CoA reductase, low-density lipoprotein (LDL) receptor and LDL receptor-related protein-1 were expectedly downregulated, especially at 8 and 14 weeks, suggesting that cholesterol synthesis and uptake in response to cholesterol accumulation may not be disorganized. The HFC diet did not upregulate liver X receptor-α, conversely, it enhanced classic BA synthesis by upregulating cholesterol 7α-hydroxylase but downregulating sterol 12α-hydroxylase, and influenced alternative synthesis by downregulating sterol 27-hydroxylase but upregulating oxysterol 7α-hydroxylase, mainly at 8 and 14 weeks, indicating that there were different productions of primary BA species. Unexpectedly, no feedback inhibition of BA synthesis by farnesoid X receptor occurred. Additionally, the HFC diet impaired BA detoxification by UDP-glucuronosyltransferase and sulfotransferase 2A1, and decreased excretion by bile salt export pump at 8 and 14 weeks, although it induced compensatory export by multidrug resistance-associated protein-3. The disturbed BA detoxification may correlate with suppressed pregnane X receptor and constitutive androstane receptor.
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