Copper is a trace element indispensable for life, but at the same time it is implicated in reactive oxygen species formation. Several inherited copper storage diseases are described of which Wilson disease (copper overload, mutations in ATP7B gene) and Menkes disease (copper deficiency, mutations in ATP7A gene) are the most prominent ones. After the discovery in 2002 of a novel gene product (i.e. COMMD1) involved in hepatic copper handling in Bedlington terriers, studies on the mechanism of action of COMMD1 revealed numerous non-copper related functions. Effects on hepatic copper handling are likely mediated via interactions with ATP7B. In addition, COMMD1 has many more interacting partners which guide their routing to either the plasma membrane or, often in an ubiquitination-dependent fashion, trigger their proteolysis via the S26 proteasome. By stimulating NF-κB ubiquitination, COMMD1 dampens an inflammatory reaction. Finally, targeting COMMD1 function can be a novel approach in the treatment of tumors.

铜是生命不可或缺的微量元素，但同时也与活性氧的形成有关。描述了几种遗传性铜贮积病，其中最突出的是威尔逊病（铜过载、ATP7B基因突变）和门克斯病（铜缺乏、ATP7A基因突变）。在 2002 年发现了一种新的基因产物（即 COMMD1）参与贝灵顿梗的肝脏铜处理后，对 COMMD1 作用机制的研究揭示了许多与铜无关的功能。对肝脏铜处理的影响可能是通过与ATP7B 的相互作用来介导的。此外，COMMD1 有更多相互作用的伙伴，它们引导它们路由到质膜，或者通常以泛素化依赖的方式通过 S26 蛋白酶体触发它们的蛋白水解。通过刺激 NF-κB 泛素化，COMMD1 抑制炎症反应。最后，靶向 COMMD1 功能可以成为治疗肿瘤的新方法。