Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1

Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Neuromuscular disorders : NMD 2015-04, Vol.25 (4), p.308-320
Hauptverfasser: Algalarrondo, Vincent, Wahbi, Karim, Sebag, Frédéric, Gourdon, Geneviève, Beldjord, Chérif, Azibi, Kamel, Balse, Elise, Coulombe, Alain, Fischmeister, Rodolphe, Eymard, Bruno, Duboc, Denis, Hatem, Stéphane N
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 320
container_issue 4
container_start_page 308
container_title Neuromuscular disorders : NMD
container_volume 25
creator Algalarrondo, Vincent
Wahbi, Karim
Sebag, Frédéric
Gourdon, Geneviève
Beldjord, Chérif
Azibi, Kamel
Balse, Elise
Coulombe, Alain
Fischmeister, Rodolphe
Eymard, Bruno
Duboc, Denis
Hatem, Stéphane N
description Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7 times faster rate of INa inactivation in DMSXL myocytes. • The study points the sodium current as a major component of the rhythmic phenotype in DM1.
doi_str_mv 10.1016/j.nmd.2014.11.018
format Article
fullrecord <record><control><sourceid>proquest_hal_p</sourceid><recordid>TN_cdi_hal_primary_oai_HAL_hal_02610568v1</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>1_s2_0_S0960896614007020</els_id><sourcerecordid>1666293167</sourcerecordid><originalsourceid>FETCH-LOGICAL-c555t-c9035a191b76ffb573c73b27c09b709de76a4e2489674c4a487b4fede812d5213</originalsourceid><addsrcrecordid>eNp9ksFu1DAQhiMEokvhAbggH-GQxeMkdiIkpFUFLdJKHICz5dgT1UtiL7ZTKQ_Rd8ZRSg89cLGt0ff_1sw_RfEW6B4o8I-nvZvMnlGo9wB7Cu2zYgetqEpW8fp5saMdp2XbcX5RvIrxRCk0gouXxQVrOFQtFbvi_tA7HyY1kuiNnSei5xDQJXIO_owhWYxEe5eC7eeEJHmiVTBWaYIj6lzWWaqc2SClkx2RmCUOs8tv74h1RJHJzxHzaXAkfiDT4pN3Vq9gyv_cLiQtZyTwungxqDHim4f7svj19cvPq5vy-P3629XhWOqmaVKpO1o1CjroBR-GvhGVFlXPhKZdL2hnUHBVI6tz66LWtapb0dcDGmyBmYZBdVl82Hxv1SjPwU4qLNIrK28OR7nWKONAG97erez7jc0T-TNjTHKyUeM4Koe5LQmcc9ZVwEVGYUN18DEGHB69gco1MXmSOTG5JiYBZE4sa9492M_9hOZR8S-iDHzaAMwDubMYZNQWnUZjQ05AGm__a__5iVqP1q2p_cYF48nPweVJS5CRSSp_rCuzbgzUlArKaPUXGbO9Sg</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1666293167</pqid></control><display><type>article</type><title>Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1</title><source>MEDLINE</source><source>Elsevier ScienceDirect Journals</source><creator>Algalarrondo, Vincent ; Wahbi, Karim ; Sebag, Frédéric ; Gourdon, Geneviève ; Beldjord, Chérif ; Azibi, Kamel ; Balse, Elise ; Coulombe, Alain ; Fischmeister, Rodolphe ; Eymard, Bruno ; Duboc, Denis ; Hatem, Stéphane N</creator><creatorcontrib>Algalarrondo, Vincent ; Wahbi, Karim ; Sebag, Frédéric ; Gourdon, Geneviève ; Beldjord, Chérif ; Azibi, Kamel ; Balse, Elise ; Coulombe, Alain ; Fischmeister, Rodolphe ; Eymard, Bruno ; Duboc, Denis ; Hatem, Stéphane N</creatorcontrib><description>Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7 times faster rate of INa inactivation in DMSXL myocytes. • The study points the sodium current as a major component of the rhythmic phenotype in DM1.</description><identifier>ISSN: 0960-8966</identifier><identifier>EISSN: 1873-2364</identifier><identifier>DOI: 10.1016/j.nmd.2014.11.018</identifier><identifier>PMID: 25613807</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>Action Potentials - drug effects ; Action Potentials - physiology ; Aging ; Animals ; Anti-Arrhythmia Agents - pharmacology ; Arrhythmia basic study ; Arrhythmias, Cardiac - physiopathology ; Brugada Syndrome ; Cardiac Conduction System Disease ; Cardiology and cardiovascular system ; Computer Simulation ; Conduction disorders ; Disease Models, Animal ; Echocardiography, Doppler ; Electrophysiology ; Flecainide - pharmacology ; Heart Conduction System - abnormalities ; Heart Conduction System - physiopathology ; Human health and pathology ; Life Sciences ; Male ; Mice, Transgenic ; Microelectrodes ; Models, Cardiovascular ; Models, Neurological ; Myocytes, Cardiac - drug effects ; Myocytes, Cardiac - physiology ; Myotonic Dystrophy - genetics ; Myotonic Dystrophy - physiopathology ; Myotonic dystrophy type 1 ; NAV1.5 Voltage-Gated Sodium Channel - genetics ; Neurology ; Patch-Clamp Techniques ; Sodium - metabolism ; Sodium current channelopathy ; Steinert disease ; Transgenic mice ; Voltage-Gated Sodium Channel Blockers - pharmacology</subject><ispartof>Neuromuscular disorders : NMD, 2015-04, Vol.25 (4), p.308-320</ispartof><rights>Elsevier B.V.</rights><rights>2014 Elsevier B.V.</rights><rights>Copyright © 2014 Elsevier B.V. All rights reserved.</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c555t-c9035a191b76ffb573c73b27c09b709de76a4e2489674c4a487b4fede812d5213</citedby><cites>FETCH-LOGICAL-c555t-c9035a191b76ffb573c73b27c09b709de76a4e2489674c4a487b4fede812d5213</cites><orcidid>0000-0002-9142-1382 ; 0000-0003-2086-9865</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0960896614007020$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,314,776,780,881,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25613807$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-02610568$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Algalarrondo, Vincent</creatorcontrib><creatorcontrib>Wahbi, Karim</creatorcontrib><creatorcontrib>Sebag, Frédéric</creatorcontrib><creatorcontrib>Gourdon, Geneviève</creatorcontrib><creatorcontrib>Beldjord, Chérif</creatorcontrib><creatorcontrib>Azibi, Kamel</creatorcontrib><creatorcontrib>Balse, Elise</creatorcontrib><creatorcontrib>Coulombe, Alain</creatorcontrib><creatorcontrib>Fischmeister, Rodolphe</creatorcontrib><creatorcontrib>Eymard, Bruno</creatorcontrib><creatorcontrib>Duboc, Denis</creatorcontrib><creatorcontrib>Hatem, Stéphane N</creatorcontrib><title>Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1</title><title>Neuromuscular disorders : NMD</title><addtitle>Neuromuscul Disord</addtitle><description>Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7 times faster rate of INa inactivation in DMSXL myocytes. • The study points the sodium current as a major component of the rhythmic phenotype in DM1.</description><subject>Action Potentials - drug effects</subject><subject>Action Potentials - physiology</subject><subject>Aging</subject><subject>Animals</subject><subject>Anti-Arrhythmia Agents - pharmacology</subject><subject>Arrhythmia basic study</subject><subject>Arrhythmias, Cardiac - physiopathology</subject><subject>Brugada Syndrome</subject><subject>Cardiac Conduction System Disease</subject><subject>Cardiology and cardiovascular system</subject><subject>Computer Simulation</subject><subject>Conduction disorders</subject><subject>Disease Models, Animal</subject><subject>Echocardiography, Doppler</subject><subject>Electrophysiology</subject><subject>Flecainide - pharmacology</subject><subject>Heart Conduction System - abnormalities</subject><subject>Heart Conduction System - physiopathology</subject><subject>Human health and pathology</subject><subject>Life Sciences</subject><subject>Male</subject><subject>Mice, Transgenic</subject><subject>Microelectrodes</subject><subject>Models, Cardiovascular</subject><subject>Models, Neurological</subject><subject>Myocytes, Cardiac - drug effects</subject><subject>Myocytes, Cardiac - physiology</subject><subject>Myotonic Dystrophy - genetics</subject><subject>Myotonic Dystrophy - physiopathology</subject><subject>Myotonic dystrophy type 1</subject><subject>NAV1.5 Voltage-Gated Sodium Channel - genetics</subject><subject>Neurology</subject><subject>Patch-Clamp Techniques</subject><subject>Sodium - metabolism</subject><subject>Sodium current channelopathy</subject><subject>Steinert disease</subject><subject>Transgenic mice</subject><subject>Voltage-Gated Sodium Channel Blockers - pharmacology</subject><issn>0960-8966</issn><issn>1873-2364</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9ksFu1DAQhiMEokvhAbggH-GQxeMkdiIkpFUFLdJKHICz5dgT1UtiL7ZTKQ_Rd8ZRSg89cLGt0ff_1sw_RfEW6B4o8I-nvZvMnlGo9wB7Cu2zYgetqEpW8fp5saMdp2XbcX5RvIrxRCk0gouXxQVrOFQtFbvi_tA7HyY1kuiNnSei5xDQJXIO_owhWYxEe5eC7eeEJHmiVTBWaYIj6lzWWaqc2SClkx2RmCUOs8tv74h1RJHJzxHzaXAkfiDT4pN3Vq9gyv_cLiQtZyTwungxqDHim4f7svj19cvPq5vy-P3629XhWOqmaVKpO1o1CjroBR-GvhGVFlXPhKZdL2hnUHBVI6tz66LWtapb0dcDGmyBmYZBdVl82Hxv1SjPwU4qLNIrK28OR7nWKONAG97erez7jc0T-TNjTHKyUeM4Koe5LQmcc9ZVwEVGYUN18DEGHB69gco1MXmSOTG5JiYBZE4sa9492M_9hOZR8S-iDHzaAMwDubMYZNQWnUZjQ05AGm__a__5iVqP1q2p_cYF48nPweVJS5CRSSp_rCuzbgzUlArKaPUXGbO9Sg</recordid><startdate>20150401</startdate><enddate>20150401</enddate><creator>Algalarrondo, Vincent</creator><creator>Wahbi, Karim</creator><creator>Sebag, Frédéric</creator><creator>Gourdon, Geneviève</creator><creator>Beldjord, Chérif</creator><creator>Azibi, Kamel</creator><creator>Balse, Elise</creator><creator>Coulombe, Alain</creator><creator>Fischmeister, Rodolphe</creator><creator>Eymard, Bruno</creator><creator>Duboc, Denis</creator><creator>Hatem, Stéphane N</creator><general>Elsevier B.V</general><general>Elsevier</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>1XC</scope><scope>VOOES</scope><orcidid>https://orcid.org/0000-0002-9142-1382</orcidid><orcidid>https://orcid.org/0000-0003-2086-9865</orcidid></search><sort><creationdate>20150401</creationdate><title>Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1</title><author>Algalarrondo, Vincent ; Wahbi, Karim ; Sebag, Frédéric ; Gourdon, Geneviève ; Beldjord, Chérif ; Azibi, Kamel ; Balse, Elise ; Coulombe, Alain ; Fischmeister, Rodolphe ; Eymard, Bruno ; Duboc, Denis ; Hatem, Stéphane N</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c555t-c9035a191b76ffb573c73b27c09b709de76a4e2489674c4a487b4fede812d5213</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Action Potentials - drug effects</topic><topic>Action Potentials - physiology</topic><topic>Aging</topic><topic>Animals</topic><topic>Anti-Arrhythmia Agents - pharmacology</topic><topic>Arrhythmia basic study</topic><topic>Arrhythmias, Cardiac - physiopathology</topic><topic>Brugada Syndrome</topic><topic>Cardiac Conduction System Disease</topic><topic>Cardiology and cardiovascular system</topic><topic>Computer Simulation</topic><topic>Conduction disorders</topic><topic>Disease Models, Animal</topic><topic>Echocardiography, Doppler</topic><topic>Electrophysiology</topic><topic>Flecainide - pharmacology</topic><topic>Heart Conduction System - abnormalities</topic><topic>Heart Conduction System - physiopathology</topic><topic>Human health and pathology</topic><topic>Life Sciences</topic><topic>Male</topic><topic>Mice, Transgenic</topic><topic>Microelectrodes</topic><topic>Models, Cardiovascular</topic><topic>Models, Neurological</topic><topic>Myocytes, Cardiac - drug effects</topic><topic>Myocytes, Cardiac - physiology</topic><topic>Myotonic Dystrophy - genetics</topic><topic>Myotonic Dystrophy - physiopathology</topic><topic>Myotonic dystrophy type 1</topic><topic>NAV1.5 Voltage-Gated Sodium Channel - genetics</topic><topic>Neurology</topic><topic>Patch-Clamp Techniques</topic><topic>Sodium - metabolism</topic><topic>Sodium current channelopathy</topic><topic>Steinert disease</topic><topic>Transgenic mice</topic><topic>Voltage-Gated Sodium Channel Blockers - pharmacology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Algalarrondo, Vincent</creatorcontrib><creatorcontrib>Wahbi, Karim</creatorcontrib><creatorcontrib>Sebag, Frédéric</creatorcontrib><creatorcontrib>Gourdon, Geneviève</creatorcontrib><creatorcontrib>Beldjord, Chérif</creatorcontrib><creatorcontrib>Azibi, Kamel</creatorcontrib><creatorcontrib>Balse, Elise</creatorcontrib><creatorcontrib>Coulombe, Alain</creatorcontrib><creatorcontrib>Fischmeister, Rodolphe</creatorcontrib><creatorcontrib>Eymard, Bruno</creatorcontrib><creatorcontrib>Duboc, Denis</creatorcontrib><creatorcontrib>Hatem, Stéphane N</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><collection>Hyper Article en Ligne (HAL) (Open Access)</collection><jtitle>Neuromuscular disorders : NMD</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Algalarrondo, Vincent</au><au>Wahbi, Karim</au><au>Sebag, Frédéric</au><au>Gourdon, Geneviève</au><au>Beldjord, Chérif</au><au>Azibi, Kamel</au><au>Balse, Elise</au><au>Coulombe, Alain</au><au>Fischmeister, Rodolphe</au><au>Eymard, Bruno</au><au>Duboc, Denis</au><au>Hatem, Stéphane N</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1</atitle><jtitle>Neuromuscular disorders : NMD</jtitle><addtitle>Neuromuscul Disord</addtitle><date>2015-04-01</date><risdate>2015</risdate><volume>25</volume><issue>4</issue><spage>308</spage><epage>320</epage><pages>308-320</pages><issn>0960-8966</issn><eissn>1873-2364</eissn><abstract>Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7 times faster rate of INa inactivation in DMSXL myocytes. • The study points the sodium current as a major component of the rhythmic phenotype in DM1.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>25613807</pmid><doi>10.1016/j.nmd.2014.11.018</doi><tpages>13</tpages><orcidid>https://orcid.org/0000-0002-9142-1382</orcidid><orcidid>https://orcid.org/0000-0003-2086-9865</orcidid><oa>free_for_read</oa></addata></record>
fulltext fulltext
identifier ISSN: 0960-8966
ispartof Neuromuscular disorders : NMD, 2015-04, Vol.25 (4), p.308-320
issn 0960-8966
1873-2364
language eng
recordid cdi_hal_primary_oai_HAL_hal_02610568v1
source MEDLINE; Elsevier ScienceDirect Journals
subjects Action Potentials - drug effects
Action Potentials - physiology
Aging
Animals
Anti-Arrhythmia Agents - pharmacology
Arrhythmia basic study
Arrhythmias, Cardiac - physiopathology
Brugada Syndrome
Cardiac Conduction System Disease
Cardiology and cardiovascular system
Computer Simulation
Conduction disorders
Disease Models, Animal
Echocardiography, Doppler
Electrophysiology
Flecainide - pharmacology
Heart Conduction System - abnormalities
Heart Conduction System - physiopathology
Human health and pathology
Life Sciences
Male
Mice, Transgenic
Microelectrodes
Models, Cardiovascular
Models, Neurological
Myocytes, Cardiac - drug effects
Myocytes, Cardiac - physiology
Myotonic Dystrophy - genetics
Myotonic Dystrophy - physiopathology
Myotonic dystrophy type 1
NAV1.5 Voltage-Gated Sodium Channel - genetics
Neurology
Patch-Clamp Techniques
Sodium - metabolism
Sodium current channelopathy
Steinert disease
Transgenic mice
Voltage-Gated Sodium Channel Blockers - pharmacology
title Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-02T16%3A22%3A00IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_hal_p&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Abnormal%20sodium%20current%20properties%20contribute%20to%20cardiac%20electrical%20and%20contractile%20dysfunction%20in%20a%20mouse%20model%20of%20myotonic%20dystrophy%20type%201&rft.jtitle=Neuromuscular%20disorders%20:%20NMD&rft.au=Algalarrondo,%20Vincent&rft.date=2015-04-01&rft.volume=25&rft.issue=4&rft.spage=308&rft.epage=320&rft.pages=308-320&rft.issn=0960-8966&rft.eissn=1873-2364&rft_id=info:doi/10.1016/j.nmd.2014.11.018&rft_dat=%3Cproquest_hal_p%3E1666293167%3C/proquest_hal_p%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1666293167&rft_id=info:pmid/25613807&rft_els_id=1_s2_0_S0960896614007020&rfr_iscdi=true