Bile Acid Synthesis. Bile acid synthesis is the predominant metabolic pathway for catabolism of cholesterol in humans. 41 bile acid synthesis 1 bile acids are synthesised from cholesterol 2 two.
Bile acid synthesis, signaling, and regulation in human
1 is an abbreviated version of this complex metabolic pathway. The classic bile acid synthesis pathway consists of a cascade of fourteen steps catalyzed by enzymes located in the cytoplasm, microsomes, mitochondria and peroxisomes. The synthesis of bile acids in the liver is regulated by a negative feedback mechanism from the bile acids reabsorbed from the intestine.
Bile acid synthesis, signaling, and regulation in human
Studies in patients with a peroxisomal disorder have been indispensable for the elucidation of the precise role of peroxisomes. Bile acid synthesis disorders due to single enzyme defects are a group of rare genetic disorders that affect the enzymes responsible for making normal, healthy bile acids. Cyp7a1 is a member of the cytochrome p450 family of metabolic enzymes. These reactions mainly occur within specialized liver cells known as hepatocytes.