Key result
In CPVT mice, increased RyR Ca2+ leak caused a depolarizing shift in activation and a hyperpolarizing shift in inactivation of cardiac L-type Ca2+ channels, reducing the Ca2+ window current.
p-value: p=<0.05
Increased RyR Ca2+ leak limits cardiac Ca2+ window current by altering LTCC gating, revealing a nanodomain Ca2+ control mechanism that overcomes the tonic effect of calmodulin.
Reveals potential compensatory LTCC gating in CPVT mice; hypothesis-generating and should not yet inform clinical practice.
Ca(2+) mediates the functional coupling between L-type Ca(2+) channel (LTCC) and sarcoplasmic reticulum (SR) Ca(2+) release channel (ryanodine receptor, RyR), participating in key pathophysiological processes. This crosstalk manifests as the orthograde Ca(2+)-induced Ca(2+)-release (CICR) mechanism triggered by Ca(2+) influx, but also as the retrograde Ca(2+)-dependent inactivation (CDI) of LTCC, which depends on both Ca(2+) permeating through the LTCC itself and on SR Ca(2+) release through the RyR. This latter effect has been suggested to rely on local rather than global Ca(2+) signaling, which might parallel the nanodomain control of CDI carried out through calmodulin (CaM). Analyzing the CICR in catecholaminergic polymorphic ventricular tachycardia (CPVT) mice as a model of RyR-generated Ca(2+) leak, we evidence here that increased occurrence of the discrete local SR Ca(2+) releases through the RyRs (Ca(2+) sparks) cause a depolarizing shift in activation and a hyperpolarizing shift in isochronic inactivation of cardiac LTCC current resulting in the reduction of window current. Both increasing fast [Ca(2+)](i) buffer capacity or depleting SR Ca(2+) store blunted these changes, which could be reproduced in WT cells by RyRCa(2+) leak induced with Ryanodol and CaM inhibition.Our results unveiled a new paradigm for CaM-dependent effect on LTCC gating and further the nanodomain Ca(2+) control of LTCC, emphasizing the importance of spatio-temporal relationships between Ca(2+) signals and CaM function.
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Fernández‐Velasco et al. (2011) studied Catecholaminergic polymorphic ventricular tachycardia (CPVT) model. RyR R4496C mutation (CPVT model) vs. Wild-type (WT) littermates was evaluated on Ca2+ window current (IWCa) and voltage-dependent activation/inactivation of L-type Ca2+ channels (p=<0.05). In CPVT mice, increased RyR Ca2+ leak caused a depolarizing shift in activation and a hyperpolarizing shift in inactivation of cardiac L-type Ca2+ channels, reducing the Ca2+ window current.
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