Bile Acid Synthesis Defect and Hyperinsulinism
نویسندگان
چکیده
منابع مشابه
Bile Acid Synthesis
During biosynthesis of bile acid, carbons 25-26-27 are removed from the cholesterol side-chain. Side-chain oxidation begins either with hydroxylation at the 26-position, in which case the three-carbon fragment is released as propionic acid, or with hydroxylation at the 25-position, in which case the threecarbon fragment is released as acetone. We have previously shown in the rat that the contri...
متن کاملBile Acid Synthesis
f Abstract The synthesis and excretion of bile acids comprise the major pathway of cholesterol catabolism in mammals. Synthesis provides a direct means of converting cholesterol, which is both hydrophobic and insoluble, into a water-soluble and readily excreted molecule, the bile acid. The biosynthetic steps that accomplish this transformation also confer detergent properties to the bile acid, ...
متن کاملRegulation of bile acid synthesis.
Bile acids are important physiological agents required for disposal of cholesterol and absorption of vitamins and fats. Bile acids are synthesized from cholesterol in the liver. Enterohepatic circulation of bile acids is very efficient and plays an important physiological role in lipid absorption and secretion, and regulation of bile acid biosynthesis and cholesterol homeostasis. Conversion of ...
متن کاملBile acid synthesis in humans.
Metabolic pathways involved in the conversion of cholesterol to cholic and chenodeoxycholic acids have been investigated in bile fistula patients treated with a number of labeled potential bile acid intermediates. The findings of the present report indicate that the human liver cell has the capacity to synthesize both primary bile acids via multiple routes from cholesterol and 7 alpha-hydroxych...
متن کاملElevated Cholesterol and Bile Acid Synthesis
outputs in the normal child were 11.5 and 3.3 mg/kg per d, respectively. Thus, in comparison with the normal child, the patient with homozygous familial hypercholesterolemia had a marked increase in synthesis of cholesterol and bile acids. Although synthesis ofbile acids was high in this patient, the fraction of newly synthesized cholesterol converted into bile acids (4056%) was in the normal r...
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ژورنال
عنوان ژورنال: ACG Case Reports Journal
سال: 2018
ISSN: 2326-3253
DOI: 10.14309/02075970-201805000-00089