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Identification of 3 alpha, 6 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholestanoic acid in Zellweger's syndrome.

Abstract
In order to confirm the occurrence of 3 alpha, 6 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholestanoic acid in Zellweger's syndrome, the nature of tetrahydroxycholestanoic acids present in a patient with this disease was studied. Urinary bile acids were extracted with a Sep-pak C18 cartridge and methylated after alkaline hydrolysis. The methyl esters were purified by silica gel column chromatography, and the methyl tetrahydroxycholestanoate fraction was analyzed by gas liquid chromatography-mass spectrometry. Along with already known side chain hydroxylated derivatives of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid, 3 alpha, 7 alpha, 12 alpha, 24- and 3 alpha, 7 alpha, 12 alpha, 26-tetrahydroxy-5 beta-cholestanoic acids, three nuclear hydroxylated derivatives of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid were found. One of them was identified as 3 alpha, 6 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholestanoic acid by direct comparison with the authentic standard which was chemically synthesized from 3 alpha, 6 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholanoic acid by side chain elongation.
AuthorsM Une, N Kisaka, M Yoshii, T Hoshita
JournalJournal of biochemistry (J Biochem) Vol. 106 Issue 3 Pg. 501-4 (Sep 1989) ISSN: 0021-924X [Print] England
PMID2606901 (Publication Type: Journal Article)
Chemical References
  • Bile Acids and Salts
  • Cholestanols
  • 3,6,7,12-tetrahydroxycholestanoic acid
Topics
  • Bile Acids and Salts (urine)
  • Cholestanols (chemical synthesis, urine)
  • Chromatography, Gas
  • Humans
  • Infant
  • Zellweger Syndrome (urine)

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