The main metabolic pathway for the bile acid intermediates di- and trihydroxycoprostanic acid is through peroxisomal ß-oxidation. Patients with either a peroxisome biogenesis defect or a specifically bile acid ß-oxidation defect accumulate these DHCA and THCA in blood and bile.

Measurement of DHCA and THCA in body fluids contributes to the diagnosis of such defects, both postnatal and prenatal. 


- dihydroxycoprostanic acid
- [27,27,27-2H3]dihydroxycoprostanic acid 
- trihydroxycoprostanic acid 
- [27,27,27-2H3]trihydroxycoprostanic acid 

Synthesis and application

DW Johnson, HJ ten Brink, RC Schuit, C Jakobs. Rapid and quantitative analysis of unconjugated C27 bile acids in plasma and blood samples by tandem mass spectrometry. J Lipid Res 42 (2001) 6-16.