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DOI | 10.1126/science.aat9819 |
Structure of the human PKD1-PKD2 complex | |
Su, Qiang1; Hu, Feizhuo1; Ge, Xiaofei1; Lei, Jianlin2; Yu, Shengqiang3; Wang, Tingliang1,4; Zhou, Qiang1; Mei, Chianglin3; Shi, Yigong1,4 | |
2018-09-07 | |
发表期刊 | SCIENCE
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ISSN | 0036-8075 |
EISSN | 1095-9203 |
出版年 | 2018 |
卷号 | 361期号:6406页码:992-+ |
文章类型 | Article |
语种 | 英语 |
国家 | Peoples R China |
英文摘要 | Mutations in two genes, PKD1 and PKD2, account for most cases of autosomal dominant polycystic kidney disease, one of the most common monogenetic disorders. Here we report the 3.6-angstrom cryo-electron microscopy structure of truncated human PKD1-PKD2 complex assembled in a 1: 3 ratio. PKD1 contains a voltage-gated ion channel (VGIC) fold that interacts with PKD2 to form the domain-swapped, yet noncanonical, transient receptor potential (TRP) channel architecture. The S6 helix in PKD1 is broken in the middle, with the extracellular half, S6a, resembling pore helix 1 in a typical TRP channel. Three positively charged, cavity-facing residues on S6b may block cation permeation. In addition to the VGIC, a five-transmembrane helix domain and a cytosolic PLAT domain were resolved in PKD1. The PKD1-PKD2 complex structure establishes a framework for dissecting the function and disease mechanisms of the PKD proteins. |
领域 | 地球科学 ; 气候变化 ; 资源环境 |
收录类别 | SCI-E |
WOS记录号 | WOS:000443892700039 |
WOS关键词 | POLYCYSTIC KIDNEY-DISEASE ; PKD2 MUTATIONS ; PRIMARY CILIA ; CHANNEL ; PROTEIN ; DOMAIN ; GENE ; IDENTIFICATION ; EXPRESSION ; INSIGHTS |
WOS类目 | Multidisciplinary Sciences |
WOS研究方向 | Science & Technology - Other Topics |
引用统计 | |
文献类型 | 期刊论文 |
条目标识符 | http://119.78.100.173/C666/handle/2XK7JSWQ/199521 |
专题 | 地球科学 资源环境科学 气候变化 |
作者单位 | 1.Tsinghua Univ, Beijing Adv Innovat Ctr Struct Biol, Tsinghua Peking Joint Ctr Life Sci, Sch Med,Sch Life Sci, Beijing 100084, Peoples R China; 2.Tsinghua Univ, Minist Educ Key Lab Prot Sci, Technol Ctr Prot Sci, Sch Life Sci, Beijing 100084, Peoples R China; 3.Second Mil Med Univ, Changzheng Hosp, Dept Nephrol, Shanghai 200433, Peoples R China; 4.Westlake Univ, Inst Biol, Westlake Inst Adv Study, 18 Shilongshan Rd, Hangzhou 310064, Zhejiang, Peoples R China |
推荐引用方式 GB/T 7714 | Su, Qiang,Hu, Feizhuo,Ge, Xiaofei,et al. Structure of the human PKD1-PKD2 complex[J]. SCIENCE,2018,361(6406):992-+. |
APA | Su, Qiang.,Hu, Feizhuo.,Ge, Xiaofei.,Lei, Jianlin.,Yu, Shengqiang.,...&Shi, Yigong.(2018).Structure of the human PKD1-PKD2 complex.SCIENCE,361(6406),992-+. |
MLA | Su, Qiang,et al."Structure of the human PKD1-PKD2 complex".SCIENCE 361.6406(2018):992-+. |
条目包含的文件 | 条目无相关文件。 |
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