2N7Y

NMR structure of metal-binding domain 1 of ATP7B

2N7Y の概要

• エントリーDOI: 10.2210/pdb2n7y/pdb
• 分子名称: Copper-transporting ATPase 2 (1 entity in total)
• 機能のキーワード: copper binding, hydrolase, metal binding protein
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Golgi apparatus, trans-Golgi network membrane ; Multi- pass membrane protein . Isoform 1: Golgi apparatus membrane ; Multi-pass membrane protein . Isoform 2: Cytoplasm . WND/140 kDa: Mitochondrion : P35670
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 8122.46

構造登録者

Yu, C.,Lee, W.,Dmitriev, O. (登録日: 2015-09-27, 公開日: 2016-09-28, 最終更新日: 2024-05-15)

主引用文献

Yu, C.H.,Lee, W.,Nokhrin, S.,Dmitriev, O.Y.
The Structure of Metal Binding Domain 1 of the Copper Transporter ATP7B Reveals Mechanism of a Singular Wilson Disease Mutation.
Sci Rep, 8:581-581, 2018

PubMed Abstract: Copper-transporter ATP7B maintains copper homeostasis in the human cells and delivers copper to the biosynthetic pathways for incorporation into the newly synthesized copper-containing proteins. ATP7B is a target of several hundred mutations that lead to Wilson disease, a chronic copper toxicosis. ATP7B contains a chain of six cytosolic metal-binding domains (MBDs), the first four of which (MBD1-4) are believed to be regulatory, and the last two (MBD5-6) are required for enzyme activity. We report the NMR structure of MBD1, the last unsolved metal-binding domain of ATP7B. The structure reveals the disruptive mechanism of G85V mutation, one of the very few disease causing missense mutations in the MBD1-4 region of ATP7B.

PubMed: 29330485
DOI: 10.1038/s41598-017-18951-1

実験手法

SOLUTION NMR