Blincoe C
University of Nevada Reno 89503-2722.
Comput Biol Med. 1993 Jan;23(1):49-55. doi: 10.1016/0010-4825(93)90107-c.
A digital computer simulation of copper metabolism was used to simulate human copper metabolism. The simulation agrees well with the normal data extant. Wilson's disease (hepatolenticular degeneration) and Menkes' disease (steely-hair syndrome) were simulated. Simulation of the unavailability of accumulated liver copper simulated Wilson's disease if it was assumed that the increased urinary excretion was due to induction of an enzymic mechanism for enhanced excretion. This would be consistent with the genetic defect causing only the sequestering of unavailable copper in the liver. Other genetic defects need not be present. Menkes' disease is also a genetic disease affecting the newborn. It was simulated successfully as a defect in absorption of copper from the gastrointestinal tract.
利用数字计算机对铜代谢进行模拟,以模拟人体铜代谢。该模拟结果与现有的正常数据吻合良好。对威尔逊氏病(肝豆状核变性)和门克斯病(卷发综合征)进行了模拟。如果假设尿排泄增加是由于诱导了一种增强排泄的酶机制,那么对肝脏中积累的无法利用的铜进行模拟就可以模拟威尔逊氏病。这与仅导致肝脏中无法利用的铜被隔离的基因缺陷是一致的。不需要存在其他基因缺陷。门克斯病也是一种影响新生儿的遗传病。它被成功模拟为胃肠道铜吸收缺陷。