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cholic acid primary bile cholate chenodeoxycholic bile liver cholesterol cholyl-coa coa glycine taurine glycocholic gallstones taurocholic zellweger syndrome sterol 27-hydroxylase cerebrotendinous xanthomatosis cholesterol alpha-hydroxylase deficiency cyp7a1 amacr statin pathway metabolite metabolism biosynthesis mechanism path catabolism biosynthetic intermediate biogenesis substrate precursor synthesis derivative bioprecursor metabolic endogenous contractor molecule molecular formula skeletal structural structure 3d