Structure of the side chain of the C29 dicarboxylic bile acid occurring in infants with coprostanic acidemia.
The structure of the side chain of the 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-C29 dicarboxylic bile acid occurring in body fluids of infants with coprostanic acidemia was investigated by means of mass spectrometry and nuclear magnetic resonance spectroscopy. The findings identified this bile acid as 3 alpha, 7 alpha, 12 alpha-trihydroxy-27a, 27b-dihomo-5 beta-cholestane-26,27b-dioic acid (3 alpha, 7 alpha, 12 alpha-trihydroxy-27-carboxymethyl-5 beta-cholestan-26-oic acid).
D002777:Cholestanols / Q000652:urine*; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D007223:Infant; D008052:Lipid Metabolism, Inborn Errors / Q000652:urine*; D009682:Magnetic Resonance Spectroscopy
D002777:Cholestanols; C034285:3,7,12-trihydroxy-27-carboxymethylcholestan-26-oic acid