Distinct early signaling events resulting from the expression of the PRKAG2 R302Q mutant of AMPK contribute to increased myocardial glycogen.
Identifieur interne : 000E31 ( PubMed/Curation ); précédent : 000E30; suivant : 000E32Distinct early signaling events resulting from the expression of the PRKAG2 R302Q mutant of AMPK contribute to increased myocardial glycogen.
Auteurs : Karalyn D. Folmes [Canada] ; Anita Y M. Chan ; Debby P Y. Koonen ; Thomas C. Pulinilkunnil ; István Baczk ; Beth E. Hunter ; Stephanie Thorn ; Michael F. Allard ; Robert Roberts ; Michael H. Gollob ; Peter E. Light ; Jason R B. DyckSource :
- Circulation. Cardiovascular genetics [ 1942-3268 ] ; 2009.
English descriptors
- KwdEn :
- AMP-Activated Protein Kinases (genetics), AMP-Activated Protein Kinases (metabolism), Animals, Cells, Cultured, Disease Models, Animal, Female, Gene Expression, Glycogen (metabolism), Humans, Male, Mice, Mice, Transgenic, Mutation, Missense, Myocytes, Cardiac (metabolism), Rats, Signal Transduction, Wolff-Parkinson-White Syndrome (enzymology), Wolff-Parkinson-White Syndrome (genetics).
- MESH :
- chemical , genetics : AMP-Activated Protein Kinases.
- chemical , metabolism : AMP-Activated Protein Kinases, Glycogen.
- enzymology : Wolff-Parkinson-White Syndrome.
- genetics : Wolff-Parkinson-White Syndrome.
- metabolism : Myocytes, Cardiac.
- Animals, Cells, Cultured, Disease Models, Animal, Female, Gene Expression, Humans, Male, Mice, Mice, Transgenic, Mutation, Missense, Rats, Signal Transduction.
Abstract
Humans with an R302Q mutation in AMPKgamma(2) (the PRKAG2 gene) develop a glycogen storage cardiomyopathy characterized by a familial form of Wolff-Parkinson-White syndrome and cardiac hypertrophy. This phenotype is recapitulated in transgenic mice with cardiomyocyte-restricted expression of AMPKgamma(2)R302Q. Although considerable information is known regarding the consequences of harboring the gamma(2)R302Q mutation, little is known about the early signaling events that contribute to the development of this cardiomyopathy.
DOI: 10.1161/CIRCGENETICS.108.834564
PubMed: 20031621
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Chan</name></author><author><name sortKey="Koonen, Debby P Y" sort="Koonen, Debby P Y" uniqKey="Koonen D" first="Debby P Y" last="Koonen">Debby P Y. Koonen</name></author><author><name sortKey="Pulinilkunnil, Thomas C" sort="Pulinilkunnil, Thomas C" uniqKey="Pulinilkunnil T" first="Thomas C" last="Pulinilkunnil">Thomas C. Pulinilkunnil</name></author><author><name sortKey="Baczk, Istvan" sort="Baczk, Istvan" uniqKey="Baczk I" first="István" last="Baczk">István Baczk</name></author><author><name sortKey="Hunter, Beth E" sort="Hunter, Beth E" uniqKey="Hunter B" first="Beth E" last="Hunter">Beth E. Hunter</name></author><author><name sortKey="Thorn, Stephanie" sort="Thorn, Stephanie" uniqKey="Thorn S" first="Stephanie" last="Thorn">Stephanie Thorn</name></author><author><name sortKey="Allard, Michael F" sort="Allard, Michael F" uniqKey="Allard M" first="Michael F" last="Allard">Michael F. Allard</name></author><author><name sortKey="Roberts, Robert" sort="Roberts, Robert" uniqKey="Roberts R" first="Robert" last="Roberts">Robert Roberts</name></author><author><name sortKey="Gollob, Michael H" sort="Gollob, Michael H" uniqKey="Gollob M" first="Michael H" last="Gollob">Michael H. Gollob</name></author><author><name sortKey="Light, Peter E" sort="Light, Peter E" uniqKey="Light P" first="Peter E" last="Light">Peter E. Light</name></author><author><name sortKey="Dyck, Jason R B" sort="Dyck, Jason R B" uniqKey="Dyck J" first="Jason R B" last="Dyck">Jason R B. Dyck</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2009">2009</date><idno type="RBID">pubmed:20031621</idno><idno type="pmid">20031621</idno><idno type="doi">10.1161/CIRCGENETICS.108.834564</idno><idno type="wicri:Area/PubMed/Corpus">000E31</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000E31</idno><idno type="wicri:Area/PubMed/Curation">000E31</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">000E31</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Distinct early signaling events resulting from the expression of the PRKAG2 R302Q mutant of AMPK contribute to increased myocardial glycogen.</title><author><name sortKey="Folmes, Karalyn D" sort="Folmes, Karalyn D" uniqKey="Folmes K" first="Karalyn D" last="Folmes">Karalyn D. Folmes</name><affiliation wicri:level="1"><nlm:affiliation>Cardiovascular Research Centre, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Cardiovascular Research Centre, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta</wicri:regionArea></affiliation></author><author><name sortKey="Chan, Anita Y M" sort="Chan, Anita Y M" uniqKey="Chan A" first="Anita Y M" last="Chan">Anita Y M. Chan</name></author><author><name sortKey="Koonen, Debby P Y" sort="Koonen, Debby P Y" uniqKey="Koonen D" first="Debby P Y" last="Koonen">Debby P Y. Koonen</name></author><author><name sortKey="Pulinilkunnil, Thomas C" sort="Pulinilkunnil, Thomas C" uniqKey="Pulinilkunnil T" first="Thomas C" last="Pulinilkunnil">Thomas C. Pulinilkunnil</name></author><author><name sortKey="Baczk, Istvan" sort="Baczk, Istvan" uniqKey="Baczk I" first="István" last="Baczk">István Baczk</name></author><author><name sortKey="Hunter, Beth E" sort="Hunter, Beth E" uniqKey="Hunter B" first="Beth E" last="Hunter">Beth E. Hunter</name></author><author><name sortKey="Thorn, Stephanie" sort="Thorn, Stephanie" uniqKey="Thorn S" first="Stephanie" last="Thorn">Stephanie Thorn</name></author><author><name sortKey="Allard, Michael F" sort="Allard, Michael F" uniqKey="Allard M" first="Michael F" last="Allard">Michael F. Allard</name></author><author><name sortKey="Roberts, Robert" sort="Roberts, Robert" uniqKey="Roberts R" first="Robert" last="Roberts">Robert Roberts</name></author><author><name sortKey="Gollob, Michael H" sort="Gollob, Michael H" uniqKey="Gollob M" first="Michael H" last="Gollob">Michael H. Gollob</name></author><author><name sortKey="Light, Peter E" sort="Light, Peter E" uniqKey="Light P" first="Peter E" last="Light">Peter E. Light</name></author><author><name sortKey="Dyck, Jason R B" sort="Dyck, Jason R B" uniqKey="Dyck J" first="Jason R B" last="Dyck">Jason R B. Dyck</name></author></analytic><series><title level="j">Circulation. Cardiovascular genetics</title><idno type="eISSN">1942-3268</idno><imprint><date when="2009" type="published">2009</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>AMP-Activated Protein Kinases (genetics)</term><term>AMP-Activated Protein Kinases (metabolism)</term><term>Animals</term><term>Cells, Cultured</term><term>Disease Models, Animal</term><term>Female</term><term>Gene Expression</term><term>Glycogen (metabolism)</term><term>Humans</term><term>Male</term><term>Mice</term><term>Mice, Transgenic</term><term>Mutation, Missense</term><term>Myocytes, Cardiac (metabolism)</term><term>Rats</term><term>Signal Transduction</term><term>Wolff-Parkinson-White Syndrome (enzymology)</term><term>Wolff-Parkinson-White Syndrome (genetics)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>AMP-Activated Protein Kinases</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>AMP-Activated Protein Kinases</term><term>Glycogen</term></keywords><keywords scheme="MESH" qualifier="enzymology" xml:lang="en"><term>Wolff-Parkinson-White Syndrome</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Wolff-Parkinson-White Syndrome</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Myocytes, Cardiac</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Animals</term><term>Cells, Cultured</term><term>Disease Models, Animal</term><term>Female</term><term>Gene Expression</term><term>Humans</term><term>Male</term><term>Mice</term><term>Mice, Transgenic</term><term>Mutation, Missense</term><term>Rats</term><term>Signal Transduction</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Humans with an R302Q mutation in AMPKgamma(2) (the PRKAG2 gene) develop a glycogen storage cardiomyopathy characterized by a familial form of Wolff-Parkinson-White syndrome and cardiac hypertrophy. This phenotype is recapitulated in transgenic mice with cardiomyocyte-restricted expression of AMPKgamma(2)R302Q. Although considerable information is known regarding the consequences of harboring the gamma(2)R302Q mutation, little is known about the early signaling events that contribute to the development of this cardiomyopathy.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">20031621</PMID><DateCreated><Year>2009</Year><Month>12</Month><Day>24</Day></DateCreated><DateCompleted><Year>2010</Year><Month>03</Month><Day>23</Day></DateCompleted><DateRevised><Year>2010</Year><Month>10</Month><Day>08</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1942-3268</ISSN><JournalIssue CitedMedium="Internet"><Volume>2</Volume><Issue>5</Issue><PubDate><Year>2009</Year><Month>Oct</Month></PubDate></JournalIssue><Title>Circulation. Cardiovascular genetics</Title><ISOAbbreviation>Circ Cardiovasc Genet</ISOAbbreviation></Journal><ArticleTitle>Distinct early signaling events resulting from the expression of the PRKAG2 R302Q mutant of AMPK contribute to increased myocardial glycogen.</ArticleTitle><Pagination><MedlinePgn>457-66</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1161/CIRCGENETICS.108.834564</ELocationID><Abstract><AbstractText Label="BACKGROUND" NlmCategory="BACKGROUND">Humans with an R302Q mutation in AMPKgamma(2) (the PRKAG2 gene) develop a glycogen storage cardiomyopathy characterized by a familial form of Wolff-Parkinson-White syndrome and cardiac hypertrophy. This phenotype is recapitulated in transgenic mice with cardiomyocyte-restricted expression of AMPKgamma(2)R302Q. Although considerable information is known regarding the consequences of harboring the gamma(2)R302Q mutation, little is known about the early signaling events that contribute to the development of this cardiomyopathy.</AbstractText><AbstractText Label="METHODS AND RESULTS" NlmCategory="RESULTS">To distinguish the direct effects of gamma(2)R302Q expression from later compensatory alterations in signaling, we used transgenic mice expressing either the wild-type AMPKgamma(2) subunit (TGgamma(2)WT) or the mutated form (TGgamma(2)R302Q), in combination with acute expression of these proteins in neonatal rat cardiomyocytes. Although acute expression of gamma(2)R302Q induces AMPK activation and upregulation of glycogen synthase and AS160, with an associated increase in glycogen content, AMPK activity, glycogen synthase activity, and AS160 expression are reduced in hearts from TGgamma(2)R302Q mice, likely in response to the existing 37-fold increase in glycogen. Interestingly, gamma(2)WT expression has similar, yet less marked effects than gamma(2)R302Q expression in both cardiomyocytes and hearts.</AbstractText><AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">Using acute and chronic models of gamma(2)R302Q expression, we have differentiated the direct effects of the gamma(2)R302Q mutation from eventual compensatory modifications. Our data suggest that expression of gamma(2)R302Q induces AMPK activation and the eventual increase in glycogen content, a finding that is masked in hearts from transgenic adult mice. These findings are the first to highlight temporal differences in the effects of the PRKAG2 R302Q mutation on cardiac metabolic signaling events.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Folmes</LastName><ForeName>Karalyn D</ForeName><Initials>KD</Initials><AffiliationInfo><Affiliation>Cardiovascular Research Centre, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Chan</LastName><ForeName>Anita Y M</ForeName><Initials>AY</Initials></Author><Author ValidYN="Y"><LastName>Koonen</LastName><ForeName>Debby P Y</ForeName><Initials>DP</Initials></Author><Author ValidYN="Y"><LastName>Pulinilkunnil</LastName><ForeName>Thomas C</ForeName><Initials>TC</Initials></Author><Author ValidYN="Y"><LastName>Baczkó</LastName><ForeName>István</ForeName><Initials>I</Initials></Author><Author ValidYN="Y"><LastName>Hunter</LastName><ForeName>Beth E</ForeName><Initials>BE</Initials></Author><Author ValidYN="Y"><LastName>Thorn</LastName><ForeName>Stephanie</ForeName><Initials>S</Initials></Author><Author ValidYN="Y"><LastName>Allard</LastName><ForeName>Michael F</ForeName><Initials>MF</Initials></Author><Author ValidYN="Y"><LastName>Roberts</LastName><ForeName>Robert</ForeName><Initials>R</Initials></Author><Author ValidYN="Y"><LastName>Gollob</LastName><ForeName>Michael H</ForeName><Initials>MH</Initials></Author><Author ValidYN="Y"><LastName>Light</LastName><ForeName>Peter E</ForeName><Initials>PE</Initials></Author><Author ValidYN="Y"><LastName>Dyck</LastName><ForeName>Jason R B</ForeName><Initials>JR</Initials></Author></AuthorList><Language>eng</Language><GrantList CompleteYN="Y"><Grant><Agency>Canadian Institutes of Health Research</Agency><Country>Canada</Country></Grant></GrantList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2009</Year><Month>07</Month><Day>15</Day></ArticleDate></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Circ Cardiovasc Genet</MedlineTA><NlmUniqueID>101489144</NlmUniqueID><ISSNLinking>1942-3268</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>9005-79-2</RegistryNumber><NameOfSubstance UI="D006003">Glycogen</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 2.7.11.1</RegistryNumber><NameOfSubstance UI="D055372">AMP-Activated Protein Kinases</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 2.7.11.1</RegistryNumber><NameOfSubstance UI="C526223">PRKAG2 protein, human</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D055372" MajorTopicYN="N">AMP-Activated Protein Kinases</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="Y">genetics</QualifierName><QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000818" MajorTopicYN="N">Animals</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002478" MajorTopicYN="N">Cells, Cultured</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004195" MajorTopicYN="N">Disease Models, Animal</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D015870" MajorTopicYN="Y">Gene Expression</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006003" MajorTopicYN="N">Glycogen</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D051379" MajorTopicYN="N">Mice</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008822" MajorTopicYN="N">Mice, Transgenic</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D020125" MajorTopicYN="Y">Mutation, Missense</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D032383" MajorTopicYN="N">Myocytes, Cardiac</DescriptorName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D051381" MajorTopicYN="N">Rats</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D015398" MajorTopicYN="Y">Signal Transduction</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D014927" MajorTopicYN="N">Wolff-Parkinson-White Syndrome</DescriptorName><QualifierName UI="Q000201" MajorTopicYN="Y">enzymology</QualifierName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2009</Year><Month>12</Month><Day>25</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2009</Year><Month>12</Month><Day>25</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2010</Year><Month>3</Month><Day>24</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">20031621</ArticleId><ArticleId IdType="pii">CIRCGENETICS.108.834564</ArticleId><ArticleId IdType="doi">10.1161/CIRCGENETICS.108.834564</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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