Journal of Lipid Research, Vol 30, 541-547, Copyright © 1989 by Lipid Research, Inc.
Identification of (22R)-3 alpha,7 alpha,12 alpha,22- and (23R)-3 alpha,7 alpha,12 alpha,23-tetrahydroxy-5 beta-cholestanoic acids in urine from a patient with Zellweger's syndrome
M Une, K Tsujimura, K Kihira and T Hoshita
Institute of Pharmaceutical Sciences, Hiroshima University School of Medicine, Japan.
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.
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