Article
FKBP12.6 deficiency and defective calcium release channel (ryanodine receptor) function linked to exercise-induced sudden cardiac death.
Cell - 27 Jun 2003
Wehrens Xander H T, Lehnart Stephan E, Huang Fannie, Vest John A, Reiken Steven R, Mohler Peter J, Sun Jie, Guatimosim Silvia, Song Long Sheng, Rosemblit Nora, D'Armiento Jeanine M, Napolitano Carlo, Memmi Mirella, Priori Silvia G, Lederer W J, Marks Andrew R
Abstract excerpt
Arrhythmias, a common cause of sudden cardiac death, can occur in structurally normal hearts, although the mechanism is not known. In cardiac muscle, the ryanodine receptor (RyR2) on the sarcoplasmic reticulum releases the calcium required for muscle contraction. The FK506 binding protein (FKBP12.6) stabilizes RyR2, preventing aberrant activation of the channel during the resting phase of the cardiac cycle. We...
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