- EMDB-35628: Structure of ATP7B C983S/C985S/D1027A mutant with AMP-PNP -
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基本情報
登録情報
データベース: EMDB / ID: EMD-35628
タイトル
Structure of ATP7B C983S/C985S/D1027A mutant with AMP-PNP
マップデータ
試料
複合体: Complex of ATP7B and ANP
タンパク質・ペプチド: Copper-transporting ATPase 2
リガンド: PHOSPHOAMINOPHOSPHONIC ACID-ADENYLATE ESTER
キーワード
P-type Cu(+) transporter / Translocase
機能・相同性
機能・相同性情報
protein maturation by copper ion transfer / P-type divalent copper transporter activity / copper ion transmembrane transporter activity / P-type monovalent copper transporter activity / P-type Cu+ transporter / sequestering of calcium ion / copper ion export / copper ion import / copper ion transport / xenobiotic detoxification by transmembrane export across the plasma membrane ...protein maturation by copper ion transfer / P-type divalent copper transporter activity / copper ion transmembrane transporter activity / P-type monovalent copper transporter activity / P-type Cu+ transporter / sequestering of calcium ion / copper ion export / copper ion import / copper ion transport / xenobiotic detoxification by transmembrane export across the plasma membrane / intracellular zinc ion homeostasis / response to copper ion / Ion transport by P-type ATPases / intracellular copper ion homeostasis / monoatomic ion transmembrane transport / lactation / trans-Golgi network membrane / establishment of localization in cell / late endosome / copper ion binding / Golgi membrane / Golgi apparatus / ATP hydrolysis activity / mitochondrion / ATP binding / membrane / plasma membrane 類似検索 - 分子機能
National Natural Science Foundation of China (NSFC)
31870724
中国
National Natural Science Foundation of China (NSFC)
81125009
中国
引用
ジャーナル: Cell Rep / 年: 2023 タイトル: Structures of the human Wilson disease copper transporter ATP7B. 著者: Guo-Min Yang / Lingyi Xu / Rou-Min Wang / Xin Tao / Zi-Wei Zheng / Shenghai Chang / Demin Ma / Cheng Zhao / Yi Dong / Shan Wu / Jiangtao Guo / Zhi-Ying Wu / 要旨: The P-type ATPase ATP7B exports cytosolic copper and plays an essential role in the regulation of cellular copper homeostasis. Mutants of ATP7B cause Wilson disease (WD), an autosomal recessive ...The P-type ATPase ATP7B exports cytosolic copper and plays an essential role in the regulation of cellular copper homeostasis. Mutants of ATP7B cause Wilson disease (WD), an autosomal recessive disorder of copper metabolism. Here, we present cryoelectron microscopy (cryo-EM) structures of human ATP7B in the E1 state in the apo, the putative copper-bound, and the putative cisplatin-bound forms. In ATP7B, the N-terminal sixth metal-binding domain (MBD6) binds at the cytosolic copper entry site of the transmembrane domain (TMD), facilitating the delivery of copper from the MBD6 to the TMD. The sulfur-containing residues in the TMD of ATP7B mark the copper transport pathway. By comparing structures of the E1 state human ATP7B and E2-P state frog ATP7B, we propose the ATP-driving copper transport model of ATP7B. These structures not only advance our understanding of the mechanisms of ATP7B-mediated copper export but can also guide the development of therapeutics for the treatment of WD.