Wenk G, Suzuki K
J Neurochem. 1983 Dec;41(6):1648-52. doi: 10.1111/j.1471-4159.1983.tb00876.x.
The mottled (Mo) mouse is an animal model of the human congenital copper (Cu) deficiency disorder, Menkes' kinky hair syndrome. Intraperitoneal Cu chloride injections have been shown to produce clinical and morphological improvements in this mutant mouse. Cu injections (10 micrograms/g) on postnatal days 7 and 10 are shown to increase endogenous activity of the Cu-dependent enzyme dopamine-beta-hydroxylase in the brains of Mo mice. The present study provides insight into the long-term neurochemical changes resulting from a possible treatment regimen for Menkes' kinky hair syndrome.
斑驳（Mo）小鼠是人类先天性铜（Cu）缺乏症——门克斯卷发综合征的动物模型。已证明腹腔注射氯化铜可使这种突变小鼠的临床症状和形态得到改善。在出生后第7天和第10天注射铜（10微克/克）可提高Mo小鼠大脑中铜依赖性酶多巴胺-β-羟化酶的内源性活性。本研究深入探讨了针对门克斯卷发综合征可能的治疗方案所导致的长期神经化学变化。
