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dc.creatorGYÖRKE,SANDOR
dc.creatorGYÖRKE,INNA
dc.creatorTERENTYEV,DMITRY
dc.creatorVIATCHENKO-KARPINSKI,SERGE
dc.creatorWILLIAMS,SIMON C
dc.date2004-01-01
dc.date.accessioned2019-05-02T21:21:20Z
dc.date.available2019-05-02T21:21:20Z
dc.identifierhttps://scielo.conicyt.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400014
dc.identifier.urihttp://revistaschilenas.uchile.cl/handle/2250/81546
dc.descriptionCalsequestrin (CASQ2) is a high capacity Ca-binding protein expressed inside the sarcoplasmic reticulum (SR). Mutations in the cardiac calsequestrin gene (CASQ2) have been linked to arrhythmias and sudden death induced by exercise and emotional stress. We have studied the function of CASQ2 and the consequences of arrhythmogenic CASQ2 mutations on intracellular Ca signalling using a combination of approaches of reverse genetics and cellular physiology in adult cardiac myocytes. We have found that CASQ2 is an essential determinant of the ability of the SR to store and release Ca2+ in cardiac muscle. CASQ2 serves as a reservoir for Ca2+ that is readily accessible for Ca2+-induced Ca2+ release (CICR) and also as an active Ca2+ buffer that modulates the local luminal Ca-dependent closure of the SR Ca2+ release channels. At the same time, CASQ2 stabilizes the CICR process by slowing the functional recharging of SR Ca2+ stores. Abnormal restitution of the Ca2+ release channels from a luminal Ca-dependent refractory state could account for ventricular arrhythmias associated with mutations in the CASQ2 gene.
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dc.languageen
dc.publisherSociedad de Biología de Chile
dc.relation10.4067/S0716-97602004000400014
dc.rightsinfo:eu-repo/semantics/openAccess
dc.sourceBiological Research v.37 n.4 2004
dc.subjectExcitation-contraction coupling
dc.subjectcalcium-induced calcium release
dc.subjectryanodine receptor
dc.subjectcalsequestrin
dc.subjectarrhythmia
dc.titleModulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes


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