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Journal of Lipid Research, Vol. 42, 9-16, January 2001
Copyright © 2001 by Lipid Research, Inc.

Original Article

Rapid and quantitative analysis of unconjugated C₂₇ bile acids in plasma and blood samples by tandem mass spectrometry

Correspondence to: D. W. Johnson, To whom correspondence should be addressed., djohnson{at}medicine.adelaide.edu.au (E-mail)

A subgroup of peroxisomal disorders, peroxisome biogenesis defects (PBD), can be differentiated by elevated levels of C₂₇ bile acids in plasma and bile. Patients with peroxisomal disorders, who lack the ability to chain-shorten the C₂₇ bile acid intermediates into C₂₄ bile acids, show elevated levels of C₂₇ bile acids, notably 3,7-dihydroxy-5ß-cholest-26-oic acid and 3,7,12-trihydroxy-5ß-cholestan-26-oic acid. C₂₇ bile acids are normally estimated against other bile acid standards, by time-consuming gas chromatography-mass spectrometry and liquid chromatography-tandem mass spectrometry methods, in plasma (minimum of 50 µl). In this article we describe the quantitation of unconjugated di- and trihydroxy C₂₇ bile acids in 5-µl plasma samples and 3-mm blood spots, using deuterium-labeled internal standards. The synthesis of ²H₃-labeled di- and trihydroxycoprostanic acids is described. The sample preparation and analysis by electrospray tandem mass spectrometry (ES-MS/MS) takes less than 1 h and features dimethylaminoethyl ester derivatives. The levels of the di- and trihydroxy bile acids are significantly higher in PBD patients than in age-matched control subjects for both plasma and blood spots collected at birth (some stored for up to 18 years). Excellent correlation is observed between the C_26:0/C_22:0 very long chain fatty acid (VLCFA) ratio and the levels of trihydroxy C₂₇ bile acids in plasma from PBD patients.

The ES-MS/MS method can be used to rapidly screen for PBD patients in plasma samples with elevated C_26:0/C_22:0 VLCFA ratios and in archived collections of neonatal blood spots. — Johnson, D. W., H. J. ten Brink, R. C. Schuit, and C. Jakobs. Rapid and quantitive analysis of unconjugated C₂₇ bile acids in plasma and blood samples by tandem mass spectrometry. J. Lipid Res. 2001. 42: 9;–16.

Supplementary key words: peroxisome, ES-MS/MS, blood spots

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