Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes
Calsequestrin (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 c...
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Sociedad de Biología de Chile
2004
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oai:scielo:S0716-976020040004000142005-06-02Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytesGYÖRKE,SANDORGYÖRKE,INNATERENTYEV,DMITRYVIATCHENKO-KARPINSKI,SERGEWILLIAMS,SIMON C Excitation-contraction coupling calcium-induced calcium release ryanodine receptor calsequestrin arrhythmia Calsequestrin (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.info:eu-repo/semantics/openAccessSociedad de Biología de ChileBiological Research v.37 n.4 20042004-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400014en10.4067/S0716-97602004000400014 |
institution |
Scielo Chile |
collection |
Scielo Chile |
language |
English |
topic |
Excitation-contraction coupling calcium-induced calcium release ryanodine receptor calsequestrin arrhythmia |
spellingShingle |
Excitation-contraction coupling calcium-induced calcium release ryanodine receptor calsequestrin arrhythmia GYÖRKE,SANDOR GYÖRKE,INNA TERENTYEV,DMITRY VIATCHENKO-KARPINSKI,SERGE WILLIAMS,SIMON C Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
description |
Calsequestrin (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. |
author |
GYÖRKE,SANDOR GYÖRKE,INNA TERENTYEV,DMITRY VIATCHENKO-KARPINSKI,SERGE WILLIAMS,SIMON C |
author_facet |
GYÖRKE,SANDOR GYÖRKE,INNA TERENTYEV,DMITRY VIATCHENKO-KARPINSKI,SERGE WILLIAMS,SIMON C |
author_sort |
GYÖRKE,SANDOR |
title |
Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
title_short |
Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
title_full |
Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
title_fullStr |
Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
title_full_unstemmed |
Modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
title_sort |
modulation of sarcoplasmic reticulum calcium release by calsequestrin in cardiac myocytes |
publisher |
Sociedad de Biología de Chile |
publishDate |
2004 |
url |
http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400014 |
work_keys_str_mv |
AT gyorkesandor modulationofsarcoplasmicreticulumcalciumreleasebycalsequestrinincardiacmyocytes AT gyorkeinna modulationofsarcoplasmicreticulumcalciumreleasebycalsequestrinincardiacmyocytes AT terentyevdmitry modulationofsarcoplasmicreticulumcalciumreleasebycalsequestrinincardiacmyocytes AT viatchenkokarpinskiserge modulationofsarcoplasmicreticulumcalciumreleasebycalsequestrinincardiacmyocytes AT williamssimonc modulationofsarcoplasmicreticulumcalciumreleasebycalsequestrinincardiacmyocytes |
_version_ |
1718441375797608448 |