中国科学院动物研究所

论文

论文题目：	TMCO1 Is an ER Ca²⁺ Load-Activated Ca²⁺ Channel
论文题目英文：
作者：	王翘楚①，郑巧霞①，#Hai-Yan Tan①，#Bing Zhang①，李晓玲①，#Yu-Xiu Yang，#Jie Yu，#Yang Liu，#Hao Chai，王茜，#Zhong-Shuai Sun，王久强，朱姝，王凤丽，#Mao-Jun Yang，#郭彩霞，#Heng Wang，#Qing-Yin Zheng，#李扬，陈佺，#周爱民，唐铁山
论文出处：
年：	2016
卷：
期：	DOI: 10.1016/j.cell.2016.04.051
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联系作者：	#周爱民，唐铁山
发表期刊：	Cell
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论文连接	http://www.sciencedirect.com/science/article/pii/S0092867416304962
影响因子：
摘要:	Maintaining homeostasis of Ca²⁺ stores in the endoplasmic reticulum (ER) is crucial for proper Ca²⁺ signaling and key cellular functions. The Ca²⁺-release-activated Ca²⁺(CRAC) channel is responsible for Ca²⁺ influx and refilling after store depletion, but how cells cope with excess Ca²⁺ when ER stores are overloaded is unclear. We show that TMCO1 is an ER transmembrane protein that actively prevents Ca²⁺ stores from overfilling, acting as what we term a “Ca²⁺ load-activated Ca²⁺ channel” or “CLAC” channel. TMCO1 undergoes reversible homotetramerization in response to ER Ca²⁺overloading and disassembly upon Ca²⁺ depletion and forms a Ca²⁺-selective ion channel on giant liposomes. TMCO1 knockout mice reproduce the main clinical features of human cerebrofaciothoracic (CFT) dysplasia spectrum, a developmental disorder linked to TMCO1 dysfunction, and exhibit severe mishandling of ER Ca²⁺ in cells. Our findings indicate that TMCO1 provides a protective mechanism to prevent overfilling of ER stores with Ca²⁺ ions.
英文摘要：
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