美國馬薩諸塞(sai)州總醫院和哈佛(fo)醫學院等(deng)機(ji)構(gou)組成的研究(jiu)組zui近發(fa)現(xian)鈣運(yun)輸(shu)鏈中(zhong)的zui后(hou)一環：一個(ge)叫(jiao)做(zuo)EMRE的線粒體蛋白。這(zhe)一研究(jiu)發(fa)現(xian)不僅有望更深入地闡明線粒體的運(yun)作(zuo)機(ji)制，還為調(diao)查鈣運(yun)輸(shu)對于疾病的貢獻(xian)——其中(zhong)包括在生(sheng)命(ming)的頭幾個(ge)月內導致死亡的罕(han)見(jian)線粒體疾病開啟了大門(men)。相關文章在《Science》雜志上發(fa)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.