Cholate disorders

In contrast, in ileal absorptive disorders, Garbutt et al. (38) observed an increase in G T ratios of both cholate and deoxycholate, which was attributed to a decrease in enterohepatic recirculation of taurocholate and deoxycholate. It is also interesting to note that a selective conjugation of cholic acid with L-ornithine could be induced in the rat and guinea pig liver by the injection of a toxic capsular polysaccharide of Klebsiella pneumoniae (39). Partial hepatectomy has also been shown to result in a shutdown in the hepatic synthesis of glycine conjugates of bile acids (40). 

The most important substrates handled by the peroxisomal fatty acid oxidation system from the perspective of peroxisomal disorders are (1) very-long-chain fatty acids (VLCFA), notably hexacosanoic acid (C26 0), (2) pris-tanic acid (2,6,10,14-tetramethylpentadecanoic acid), as derived from dietary sources either directly or indirectly from phytanic acid and (3) di- and trihydroxycholestanoic acid (DHCA and THCA). The latter two compounds are intermediates in the formation of the primary bile acids cholate and chenodeoxycholate from cholesterol in the liver. 

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