Voskoboinik I, Brooks H, Smith S, Shen P, Camakaris J
Department of Genetics, University of Melbourne, Parkville, Vic. Australia.
FEBS Lett. 1998 Sep 18;435(2-3):178-82. doi: 10.1016/s0014-5793(98)01059-x.
The Menkes (MNK) protein is a vital component of copper homeostasis in mammalian cells. In this paper we provide the first biochemical evidence that the MNK protein functions as a copper-translocating P-type ATPase in mammalian cells. The enzyme activity in membrane vesicles prepared from Chinese hamster ovary cells overexpressing MNK was ATP-dependent, correlated with the amount of MNK and followed Michaelis-Menten kinetics with respect to copper. The copper transport was observed only under reducing conditions suggesting MNK transports Cu(I). This study opens the way to detailed structure-function studies and assessment of functional MNK derived from patients with Menkes disease.
门克斯（MNK）蛋白是哺乳动物细胞中铜稳态的重要组成部分。在本文中，我们提供了首个生化证据，证明MNK蛋白在哺乳动物细胞中作为一种铜转运P型ATP酶发挥作用。从过表达MNK的中国仓鼠卵巢细胞制备的膜囊泡中的酶活性依赖于ATP，与MNK的量相关，并且在铜方面遵循米氏动力学。仅在还原条件下观察到铜转运，表明MNK转运Cu（I）。这项研究为详细的结构功能研究以及对来自门克斯病患者的功能性MNK进行评估开辟了道路。
