Une M, Kisaka N, Yoshii M, Hoshita T
Institute of Pharmaceutical Sciences, Hiroshima University School of Medicine.
J Biochem. 1989 Sep;106(3):501-4. doi: 10.1093/oxfordjournals.jbchem.a122881.
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.
为了证实3α,6α,7α,12α-四羟基-5β-胆甾烷酸在脑肝肾综合征中的存在，对一名患有该疾病患者体内存在的四羟基胆甾烷酸的性质进行了研究。用Sep-pak C18柱提取尿胆汁酸，并在碱性水解后进行甲基化。甲酯通过硅胶柱色谱法纯化，甲基四羟基胆甾烷酸馏分通过气相色谱-质谱联用仪进行分析。除了已知的3α,7α,12α-三羟基-5β-胆甾烷酸的侧链羟基化衍生物、3α,7α,12α,24-和3α,7α,12α,26-四羟基-5β-胆甾烷酸外，还发现了3α,7α,12α-三羟基-5β-胆甾烷酸的三种核羟基化衍生物。其中一种通过与由3α,6α,7α,12α-四羟基-5β-胆烷酸经侧链延长化学合成的标准品直接比较，被鉴定为3α,6α,7α,12α-四羟基-5β-胆甾烷酸。
