ldentif ication of (22R)-3a,74 2a,22- and (23R)-3a,7&12a ,23=tet ra hyd roxy-5p-c holestanoic acids in urine from a patient with Zellweger's syndrome

The nature of two novel C2, bile acids present as the taurine conjugates in urine from a patient with Zellweger's syn- drome was studied. Bile acids conjugated with taurine were iso- lated from unconjugated and glycine-conjugated bile acids by means of ion-exchange chromatography. After alkaline hydroly- sis of the taurine conjugates, the hydrolysate was acidified and extracted with ether; the extract was again subjected to ion- exchange chromatography to separate neutral from acidic com- pounds. The neutral fraction, which consisted mainly of two steroidal lactones, was treated with lithium aluminum hydride, and the reduction products were identified as (22R)-5/.3- cholestane-3a,7a,12a,22,26-pentol and (23R)-5P-cholestane- 3a,7a,12a,23,26-pentol by direct comparison of their gas-liquid chromatographic behaviors and mass spectral data with those of chemically synthesized authentic samples. Thus, the chemical structure of two native bile acids present in urine from a patient with Zellweger's syndrome should be formulated as (22R)- 3a,7a,12a,22-tetrahydroxy-5/.3-cholestanoic acid and (23R)- 3a,7a,l2a,23-tetrahydroxy-5P-cholestanoic acid, respectively.

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