Suppr超能文献

磷酸化泛素诱导帕金森蛋白激活的机制。

Mechanism of phospho-ubiquitin-induced PARKIN activation.

作者信息

Wauer Tobias, Simicek Michal, Schubert Alexander, Komander David

机构信息

Medical Research Council Laboratory of Molecular Biology, Francis Crick Avenue, Cambridge CB2 0QH, UK.

出版信息

Nature. 2015 Aug 20;524(7565):370-4. doi: 10.1038/nature14879. Epub 2015 Jul 10.

Abstract

The E3 ubiquitin ligase PARKIN (encoded by PARK2) and the protein kinase PINK1 (encoded by PARK6) are mutated in autosomal-recessive juvenile Parkinsonism (AR-JP) and work together in the disposal of damaged mitochondria by mitophagy. PINK1 is stabilized on the outside of depolarized mitochondria and phosphorylates polyubiquitin as well as the PARKIN ubiquitin-like (Ubl) domain. These phosphorylation events lead to PARKIN recruitment to mitochondria, and activation by an unknown allosteric mechanism. Here we present the crystal structure of Pediculus humanus PARKIN in complex with Ser65-phosphorylated ubiquitin (phosphoUb), revealing the molecular basis for PARKIN recruitment and activation. The phosphoUb binding site on PARKIN comprises a conserved phosphate pocket and harbours residues mutated in patients with AR-JP. PhosphoUb binding leads to straightening of a helix in the RING1 domain, and the resulting conformational changes release the Ubl domain from the PARKIN core; this activates PARKIN. Moreover, phosphoUb-mediated Ubl release enhances Ubl phosphorylation by PINK1, leading to conformational changes within the Ubl domain and stabilization of an open, active conformation of PARKIN. We redefine the role of the Ubl domain not only as an inhibitory but also as an activating element that is restrained in inactive PARKIN and released by phosphoUb. Our work opens up new avenues to identify small-molecule PARKIN activators.

摘要

E3泛素连接酶帕金(由PARK2编码)和蛋白激酶PINK1(由PARK6编码)在常染色体隐性青少年帕金森病(AR-JP)中发生突变,并在通过线粒体自噬处理受损线粒体的过程中协同发挥作用。PINK1在去极化线粒体的外部稳定存在,并使多聚泛素以及帕金泛素样(Ubl)结构域磷酸化。这些磷酸化事件导致帕金被招募至线粒体,并通过一种未知的别构机制被激活。在此,我们展示了人虱帕金与Ser65磷酸化泛素(磷酸泛素)复合物的晶体结构,揭示了帕金被招募和激活的分子基础。帕金上的磷酸泛素结合位点包含一个保守的磷酸口袋,且含有在AR-JP患者中发生突变的残基。磷酸泛素结合导致RING1结构域中的一个螺旋伸直,所产生的构象变化使Ubl结构域从帕金核心释放;这激活了帕金。此外,磷酸泛素介导的Ubl释放增强了PINK1对Ubl的磷酸化作用,导致Ubl结构域内的构象变化以及帕金开放、活性构象的稳定。我们重新定义了Ubl结构域的作用,它不仅是一个抑制性元件,也是一个在无活性的帕金中受到抑制并被磷酸泛素释放的激活元件。我们的工作为鉴定小分子帕金激活剂开辟了新途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ac8/4984986/cc2e72a44a2d/emss-64089-f005.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验