the synthesis of primary bile acids from cholesterol occurs via two pathways: the classic neutral pathway involving cholesterol 7-alpha-hydroxylase (cyp7a1), and the acidic pathway involving a distinct microsomal oxysterol 7-alpha-hydroxylase (cyp7b1). cbasiii is characterized by accumulation of bile acids in the urine.
Parent Term
Biochemical pathway
Parent Definition
A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.
Other Metabolites Mapped to 'Congenital Bile Acid Synthesis Defect Type III'