Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?
Identifieur interne : 001573 ( Main/Corpus ); précédent : 001572; suivant : 001574Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?
Auteurs : Peter E. LightSource :
- Journal of Cardiovascular Electrophysiology [ 1045-3873 ] ; 2006-05.
English descriptors
Abstract
Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.
Url:
DOI: 10.1111/j.1540-8167.2006.00399.x
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ISTEX:B1EFACA021DBE2AB9310EA27130823FAA5530727Le document en format XML
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Light</name><affiliation><mods:affiliation>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:B1EFACA021DBE2AB9310EA27130823FAA5530727</idno><date when="2006" year="2006">2006</date><idno type="doi">10.1111/j.1540-8167.2006.00399.x</idno><idno type="url">https://api.istex.fr/document/B1EFACA021DBE2AB9310EA27130823FAA5530727/fulltext/pdf</idno><idno type="wicri:Area/Main/Corpus">001573</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title><author><name sortKey="Light, Peter E" sort="Light, Peter E" uniqKey="Light P" first="Peter E." last="Light">Peter E. Light</name><affiliation><mods:affiliation>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Journal of Cardiovascular Electrophysiology</title><idno type="ISSN">1045-3873</idno><idno type="eISSN">1540-8167</idno><imprint><publisher>Blackwell Publishing Inc</publisher><pubPlace>350 Main Street , Malden , MA 02148‐5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK .</pubPlace><date type="published" when="2006-05">2006-05</date><biblScope unit="volume">17</biblScope><biblScope unit="supplement">s1</biblScope><biblScope unit="page" from="S158">S158</biblScope><biblScope unit="page" to="S161">S161</biblScope></imprint><idno type="ISSN">1045-3873</idno></series><idno type="istex">B1EFACA021DBE2AB9310EA27130823FAA5530727</idno><idno type="DOI">10.1111/j.1540-8167.2006.00399.x</idno><idno type="ArticleID">JCE399</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1045-3873</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>arrhythmia</term><term>familiar WPW</term><term>glycogen storage</term><term>ion channels</term><term>ventricular pre‐excitation</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>PETER E. LIGHT Ph.D.</name><affiliations><json:string>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>familiar WPW</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>glycogen storage</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>ion channels</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>ventricular pre‐excitation</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>arrhythmia</value></json:item></subject><articleId><json:string>JCE399</json:string></articleId><language><json:string>eng</json:string></language><abstract>Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.</abstract><qualityIndicators><score>4.177</score><pdfVersion>1.3</pdfVersion><pdfPageSize>594.006 x 783.008 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>5</keywordCount><abstractCharCount>937</abstractCharCount><pdfWordCount>2509</pdfWordCount><pdfCharCount>16545</pdfCharCount><pdfPageCount>4</pdfPageCount><abstractWordCount>139</abstractWordCount></qualityIndicators><title>Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title><genre><json:string>article</json:string></genre><host><volume>17</volume><publisherId><json:string>JCE</json:string></publisherId><pages><total>4</total><last>S161</last><first>S158</first></pages><issn><json:string>1045-3873</json:string></issn><genre><json:string>Journal</json:string></genre><language><json:string>unknown</json:string></language><eissn><json:string>1540-8167</json:string></eissn><title>Journal of Cardiovascular Electrophysiology</title><doi><json:string>10.1111/(ISSN)1540-8167</json:string></doi></host><publicationDate>2006</publicationDate><copyrightDate>2006</copyrightDate><doi><json:string>10.1111/j.1540-8167.2006.00399.x</json:string></doi><id>B1EFACA021DBE2AB9310EA27130823FAA5530727</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/B1EFACA021DBE2AB9310EA27130823FAA5530727/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/B1EFACA021DBE2AB9310EA27130823FAA5530727/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/B1EFACA021DBE2AB9310EA27130823FAA5530727/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Blackwell Publishing Inc</publisher><pubPlace>350 Main Street , Malden , MA 02148‐5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK .</pubPlace><availability><p>WILEY</p></availability><date>2006</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title><author><persName><forename type="first">PETER E.</forename><surname>LIGHT</surname></persName><roleName type="degree">Ph.D.</roleName><affiliation>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</affiliation></author></analytic><monogr><title level="j">Journal of Cardiovascular Electrophysiology</title><idno type="pISSN">1045-3873</idno><idno type="eISSN">1540-8167</idno><idno type="DOI">10.1111/(ISSN)1540-8167</idno><imprint><publisher>Blackwell Publishing Inc</publisher><pubPlace>350 Main Street , Malden , MA 02148‐5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK .</pubPlace><date type="published" when="2006-05"></date><biblScope unit="volume">17</biblScope><biblScope unit="supplement">s1</biblScope><biblScope unit="page" from="S158">S158</biblScope><biblScope unit="page" to="S161">S161</biblScope></imprint></monogr><idno type="istex">B1EFACA021DBE2AB9310EA27130823FAA5530727</idno><idno type="DOI">10.1111/j.1540-8167.2006.00399.x</idno><idno type="ArticleID">JCE399</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2006</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>familiar WPW</term></item><item><term>glycogen storage</term></item><item><term>ion channels</term></item><item><term>ventricular pre‐excitation</term></item><item><term>arrhythmia</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2006-05">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/B1EFACA021DBE2AB9310EA27130823FAA5530727/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Blackwell Publishing Inc</publisherName><publisherLoc> 350 Main Street , Malden , MA 02148‐5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . </publisherLoc></publisherInfo><doi origin="wiley" registered="yes">10.1111/(ISSN)1540-8167</doi><issn type="print">1045-3873</issn><issn type="electronic">1540-8167</issn><idGroup><id type="product" value="JCE"></id><id type="publisherDivision" value="ST"></id></idGroup><titleGroup><title type="main" sort="JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY">Journal of Cardiovascular Electrophysiology</title></titleGroup></publicationMeta><publicationMeta level="part" position="05000"><doi origin="wiley">10.1111/jce.2006.17.issue-s1</doi><numberingGroup><numbering type="journalVolume" number="17">17</numbering><numbering type="supplement">s1</numbering></numberingGroup><coverDate startDate="2006-05">May 2006</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="24" status="forIssue"><doi origin="wiley">10.1111/j.1540-8167.2006.00399.x</doi><idGroup><id type="unit" value="JCE399"></id><id type="supplier" value="jce399"></id></idGroup><countGroup><count type="pageTotal" number="4"></count></countGroup><titleGroup><title type="tocHeading1">V. Sodium Current Involvement in Cardiovascular Disease</title><title type="tocHeading2">ORIGINAL ARTICLES</title></titleGroup><eventGroup><event type="firstOnline" date="2006-04-21"></event><event type="publishedOnlineFinalForm" date="2006-04-21"></event><event type="xmlConverted" agent="Converter:BPG_TO_WML3G version:2.3.4 mode:FullText source:FullText result:FullText" date="2010-03-30"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-01-29"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-23"></event></eventGroup><numberingGroup><numbering type="pageFirst">S158</numbering><numbering type="pageLast">S161</numbering></numberingGroup><correspondenceTo>Address for correspondence: Peter E. Light, Department of Pharmacology, 9‐58 Medical Sciences Building, University of Alberta, Edmonton, AB, Canada T6G 2H7. Fax: +1‐780‐492‐8836; E‐mail: <email>peter.light@ualberta.ca</email></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:JCE.JCE399.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="3"></count><count type="tableTotal" number="0"></count><count type="formulaTotal" number="0"></count><count type="referenceTotal" number="27"></count><count type="linksCrossRef" number="34"></count></countGroup><titleGroup><title type="main">Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title></titleGroup><creators><creator creatorRole="author" xml:id="cr1" affiliationRef="#a1"><personName><givenNames>PETER E.</givenNames><familyName>LIGHT</familyName><degrees> Ph.D. </degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="a1" countryCode="CA"><unparsedAffiliation>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en"><keyword xml:id="k1">familiar WPW</keyword><keyword xml:id="k2">glycogen storage</keyword><keyword xml:id="k3">ion channels</keyword><keyword xml:id="k4">ventricular pre‐excitation</keyword><keyword xml:id="k5">arrhythmia</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><!-- Familial WPW, Glycogen Storage, and Sodium Channels. 
<p>Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ<sub>2</sub>‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.</p><!-- (J Cardiovasc Electrophysiol, Vol. 17, pp. S158-S161, Suppl. 1, May 2006)
</abstractGroup></contentMeta><noteGroup><note xml:id="n-fnt-1" numbered="no"><p>This study was supported by funding from the Alberta Heritage Foundation for Medical Research (AHFMR), the Canadian Institutes of Health Research (CIHR), and the Heart and Stroke Foundation of Canada. Dr. Light, Ph.D., received salary support as an AHFMR Scholar and CIHR New Investigator.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Familial Wolff‐Parkinson‐White Syndrome: A Disease of Glycogen Storage or Ion Channel Dysfunction?</title></titleInfo><name type="personal"><namePart type="given">PETER E.</namePart><namePart type="family">LIGHT</namePart><namePart type="termsOfAddress">Ph.D.</namePart><affiliation>Department of Pharmacology, University of Alberta, Edmonton, Alberta, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article"></genre><originInfo><publisher>Blackwell Publishing Inc</publisher><place><placeTerm type="text">350 Main Street , Malden , MA 02148‐5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK .</placeTerm></place><dateIssued encoding="w3cdtf">2006-05</dateIssued><copyrightDate encoding="w3cdtf">2006</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">3</extent><extent unit="references">27</extent></physicalDescription><abstract lang="en">Wolff‐Parkinson‐White (WPW) syndrome is the most common cause of ventricular pre‐excitation, a condition where, due to defects in the conduction pathway, all or part of the ventricle is excited earlier than would normally be expected, often leading to ventricular fibrillation and sudden cardiac death. It was recently discovered that many of the underlying mutations responsible for the familial form of WPW syndrome are located in the gene encoding for the regulatory γ2‐subunit (PRKAG2) of the AMP‐activated protein kinase. The cellular mechanisms for the observed arrhythmias are currently being studied and may involve glycogen storage with associated hypertrophy as well as alterations in the properties of cardiac ion channels such as voltage‐gated sodium channel. It is the aim of this review to discuss our current knowledge of the cellular disturbances underlying the induction of arrhythmias in patients with PRKAG2 mutations.</abstract><subject lang="en"><genre>Keywords</genre><topic>familiar WPW</topic><topic>glycogen storage</topic><topic>ion channels</topic><topic>ventricular pre‐excitation</topic><topic>arrhythmia</topic></subject><relatedItem type="host"><titleInfo><title>Journal of Cardiovascular Electrophysiology</title></titleInfo><genre type="Journal">journal</genre><identifier type="ISSN">1045-3873</identifier><identifier type="eISSN">1540-8167</identifier><identifier type="DOI">10.1111/(ISSN)1540-8167</identifier><identifier type="PublisherID">JCE</identifier><part><date>2006</date><detail type="volume"><caption>vol.</caption><number>17</number></detail><detail type="supplement"><caption>Suppl. no.</caption><number>s1</number></detail><extent unit="pages"><start>S158</start><end>S161</end><total>4</total></extent></part></relatedItem><identifier type="istex">B1EFACA021DBE2AB9310EA27130823FAA5530727</identifier><identifier type="DOI">10.1111/j.1540-8167.2006.00399.x</identifier><identifier type="ArticleID">JCE399</identifier><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Blackwell Publishing Inc</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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