The nature of two novel C27
bile acids present as the
taurine conjugates in urine from a patient with
Zellweger's syndrome was studied.
Bile acids conjugated with
taurine were isolated from unconjugated and
glycine-conjugated
bile acids by means of ion-exchange chromatography. After alkaline hydrolysis 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 compounds. 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 beta-cholestane-3 alpha,7 alpha,12 alpha,22,26-pentol and (23R)-5 beta-cholestane-3 alpha,7 alpha,12 alpha,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)-3 alpha,7 alpha,12 alpha,22-tetrahydroxy-5 beta-cholestanoic
acid and (23R)-3 alpha,7 alpha,12 alpha,12 alpha,23-tetrahydroxy-5 beta-cholestanoic
acid, respectively.