史密斯-莱姆利-奥皮茨综合征患者中8-脱氢胆固醇（胆甾-5,8-二烯-3β-醇）的鉴定。
Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome.
作者信息
Batta A K, Tint G S, Shefer S, Abuelo D, Salen G
机构信息
Department of Medicine, University of Medicine and Dentistry-New Jersey Medical School, Newark 07103, USA.
出版信息
J Lipid Res. 1995 Apr;36(4):705-13.
Cholesta-5,8-dien-3 beta-ol (8-dehydrocholesterol) and cholesta-5,7-dien-3 beta-ol (7-dehydrocholesterol) were isolated from the fecal neutral sterol fraction from homozygotes with Smith-Lemli-Opitz syndrome. The structures of the sterols were conclusively established from their mass spectra and 1H and 13C nuclear magnetic resonance spectra. It is probable that 8-dehydrocholesterol arises from 7-dehydrocholesterol and is not a direct precursor of cholesterol.
从患有史密斯-利姆利-奥皮茨综合征的纯合子粪便中性固醇组分中分离出了胆甾-5,8-二烯-3β-醇（8-脱氢胆固醇）和胆甾-5,7-二烯-3β-醇（7-脱氢胆固醇）。通过质谱以及氢-1和碳-13核磁共振光谱最终确定了这些固醇的结构。8-脱氢胆固醇可能由7-脱氢胆固醇产生，而非胆固醇的直接前体。
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