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.
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Authors | M Une, N Kisaka, M Yoshii, T Hoshita |
Journal | Journal of biochemistry
(J Biochem)
Vol. 106
Issue 3
Pg. 501-4
(Sep 1989)
ISSN: 0021-924X [Print] England |
PMID | 2606901
(Publication Type: Journal Article)
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Chemical References |
- Bile Acids and Salts
- Cholestanols
- 3,6,7,12-tetrahydroxycholestanoic acid
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Topics |
- Bile Acids and Salts
(urine)
- Cholestanols
(chemical synthesis, urine)
- Chromatography, Gas
- Humans
- Infant
- Zellweger Syndrome
(urine)
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