Article
Localization of an NH(2)-terminal disease-causing mutation hot spot to the "clamp" region in the three-dimensional structure of the cardiac ryanodine receptor.
The Journal of biological chemistry - 15 Jun 2007
Wang Ruiwu, Chen Wenqian, Cai Shitian, Zhang Jing, Bolstad Jeff, Wagenknecht Terence, Liu Zheng, Chen S R Wayne
Abstract excerpt
A region between residues 414 and 466 in the cardiac ryanodine receptor (RyR2) harbors more than half of the known NH(2)-terminal mutations associated with cardiac arrhythmias and sudden death. To gain insight into the structural basis of this NH(2)-terminal mutation hot spot, we have determined its location in the three-dimensional structure of RyR2. Green fluorescent protein (GFP), used as a structural marker,...
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