Sensitive analysis of serum 3α, 7α, 12α,24-tetrahydroxy-5β-cholestan-26-oic acid diastereomers using gas chromatography–mass spectrometry and its application in peroxisomal d-bifunctional protein deficiency

Sensitive analysis of serum 3α, 7α, 12α,24-tetrahydroxy-5β-cholestan-26-oic acid diastereomers using gas chromatography–mass spectrometry and its application in peroxisomal d-bifunctional protein deficiency

The final steps in bile acid biosynthesis take place in peroxisomes and involve oxidative cleavage of the side chain of C27-5β-cholestanoic acids leading to the formation of the primary bile acids cholic acid and chenodeoxycholic acid. The enoyl-CoA hydratase and β-hydroxy acyl-CoA dehydrogenase rea...

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Bibliographic Details
Main Authors: P. Vreken, A. van Rooij, S. Denis, E.G. van Grunsven, D.A. Cuebas, R.J.A. Wanders
Format: Article
Language:English
Published: Elsevier 1998-12-01
Series:Journal of Lipid Research
Subjects:
peroxisome
bile acids
d-bifunctional protein
GC/MS
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520333253

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