Frommer D J
Digestion. 1977;15(5):390-6. doi: 10.1159/000198027.
The form in which copper is bound in bile was studied in bile from patients with Wilson's disease, control subjects and rats. Bile was labelled with 64Cu. Sephadex column chromatography using gels G-200--G-10 showed a major 64Cu and protein peak of about 5,000 daltons and a minor peak of about 8,000 daltons. A high-molecular-weight peak (above 800,000 daltons) which was present when the eluting solution consisted of water or saline was shown to be an artefact which could be abolished by the addition of bile salt to the eluting solution. Bile from control subjects, Wilson's disease patients and from the rats behaved in the same manner binding copper to low-molecular-weight proteins. The relationship of these findings to the pathogenesis of copper retention in Wilson's disease is discussed.
对威尔逊氏病患者、对照受试者及大鼠胆汁中铜的结合形式进行了研究。用⁶⁴Cu标记胆汁。使用G - 200 - G - 10凝胶的葡聚糖凝胶柱色谱显示出一个主要的⁶⁴Cu和蛋白质峰，分子量约为5000道尔顿，还有一个较小的峰，分子量约为8000道尔顿。当洗脱液由水或盐水组成时出现的一个高分子量峰（高于800,000道尔顿）被证明是一种假象，可通过在洗脱液中添加胆盐来消除。对照受试者、威尔逊氏病患者及大鼠的胆汁在将铜结合到低分子量蛋白质方面表现相同。讨论了这些发现与威尔逊氏病中铜潴留发病机制的关系。
