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Bile Acids

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. 

Standards
- 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.

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