Carpenter T O, Pendrak M L, Anast C S
Department of Medicine (Endocrinology), Children's Hospital, Boston, Massachusetts.
Am J Physiol. 1988 Feb;254(2 Pt 1):E150-4. doi: 10.1152/ajpendo.1988.254.2.E150.
Wilson's disease results in excess tissue accumulation of copper and is often complicated by skeletal and mineral abnormalities. We investigated vitamin D metabolism in rats fed a copper-laden diet rendering hepatic copper content comparable with that found in Wilson's disease. Injection of 25-hydroxyvitamin D3 [25(OH)D3] resulted in reduced 1,25-dihydroxyvitamin D [1,25(OH)2D] levels in copper-intoxicated rats. In vitro 25(OH)D-1 alpha-hydroxylase activity was impaired in renal mitochondria from copper-intoxicated animals. Activity was also inhibited in mitochondria from controls when copper was added to incubation media. Impaired conversion of 25(OH)D to 1,25(OH)2D occurs in copper intoxication and suggests that altered vitamin D metabolism is a potential factor in the development of bone and mineral abnormalities in Wilson's disease.
威尔逊氏病会导致铜在组织中过度蓄积，且常伴有骨骼和矿物质异常。我们研究了喂食富含铜饮食的大鼠的维生素D代谢情况，使肝脏铜含量与威尔逊氏病患者的相当。给铜中毒大鼠注射25-羟基维生素D3 [25(OH)D3]会导致1,25-二羟基维生素D [1,25(OH)2D]水平降低。铜中毒动物的肾线粒体中，体外25(OH)D-1α-羟化酶活性受损。当向孵育培养基中添加铜时，对照动物的线粒体活性也受到抑制。铜中毒时25(OH)D向1,25(OH)2D的转化受损，这表明维生素D代谢改变是威尔逊氏病中骨骼和矿物质异常发生发展的一个潜在因素。
