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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Autores principales: GYÖRKE,SANDOR, GYÖRKE,INNA, TERENTYEV,DMITRY, VIATCHENKO-KARPINSKI,SERGE, WILLIAMS,SIMON C
Lenguaje:English
Publicado: Sociedad de Biología de Chile 2004
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602004000400014
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spelling 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
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