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Science:科学家发现钙通道“线粒体蛋白EMRE”

发布时间:2013-11-25      点击次数:1471

       美国马萨诸塞州总医院和哈佛医学院等机构组成的研究组zui近发现钙运输链中的zui后一环:一个叫做EMRE的线粒体蛋白。这一研究发现不仅有望更深入地阐明线粒体的运作机制,还为调查钙运输对于疾病的贡献——其中包括在生命的头几个月内导致死亡的罕见线粒体疾病开启了大门。相关文章在《Science》杂志上发表。

原文摘要:

EMRE is an Essential Component of the Mitochondrial Calcium Uniporter Complex

Yasemin Sancak, Andrew L. Markhard, Toshi Kitami, Erika Kovács-Bogdán, Kimberli J. Kamer,Namrata D. Udeshi, Steven A. Carr, Dipayan Chaudhuri, David E. Clapham, Andrew A. Li, Sarah E. Calvo,Olga Goldberger, Vamsi K. Mootha

The mitochondrial uniporter is a highly selective calcium channel in the organelle’s inner membrane. Its molecular components include the EF-hand–containing proteins mitochondrial calcium uptake 1 (MICU1) and MICU2 and the pore-forming subunit mitochondrial calcium uniporter (MCU). We sought to achieve a full molecular characterization of the uniporter holocomplex (uniplex). Quantitative mass spectrometry of affinity-purified uniplex recovered MICU1 and MICU2, MCU and its paralog MCUb, and essential MCU regulator (EMRE), a previously uncharacterized protein. EMRE is a 10-kD, metazoan-specific protein with a single transmembrane domain. In its absence, uniporter channel activity was lost despite intact MCU expression and oligomerization. EMRE was required for the interaction of MCU with MICU1 and MICU2. Hence, EMRE is essential for in vivo uniporter current and additionally bridges the calcium-sensing role of MICU1 and MICU2 with the calcium-conducting role of MCU.

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