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A more recent version of this article appeared on November 11, 2005
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M505714200v1
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Papers In Press, published online ahead of print September 11, 2005
J. Biol. Chem, 10.1074/jbc.M505714200
Submitted on May 25, 2005
Revised on August 22, 2005
Accepted on September 11, 2005

Localization of a disease-associated mutation site on the three-dimensional structure of cardiac ryanodine receptor

Zheng Liu, Ruiwu Wang, Jing Zhang, S. R. Wayne Chen, and Terence Wagenknecht

Wadsworth Center, New York State Department of Health, Albany, NY 12201

Corresponding Author: liuz{at}wadsworth.org

The cardiac ryanodine receptor (RyR2) functions as a calcium release channel in the heart. Up to 40 mutations in RyR2 have been linked to genetic forms of sudden cardiac death. These mutations are largely clustered in three regions of the sequence of the polypeptide: one near the amino-terminus, one in the central region, and the third in the carboxy-terminal region. The central region includes 11 mutations, and an isoleucine-proline motif (2427-2428) in the same region is predicted to contribute to the binding of FKBP12.6 protein. We have mapped the central mutation site on the three-dimensional structure of RyR2, by methods of green fluorescent protein insertion, cryo-electron microscopy (cryo-EM), and single-particle image processing. The central mutation site was mapped to a “bridge” of density that connects cytoplasmic domains 5 and 6, domains that have been suggested to undergo conformational changes during channel gating. Moreover, the location of this central mutation site is not close to that of the FKBP12.6 binding site mapped previously by cryo-EM.


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