Intrinsic epigenetic regulation of the D4Z4 macrosatellite repeat in a transgenic mouse model for FSHD
Facioscapulohumeral dystrophy (FSHD) is a progressive muscular dystrophy caused by decreased epigenetic repression of the D4Z4 macrosatellite repeats and ectopic expression of DUX4, a retrogene encoding a germline transcription factor encoded in each repeat. Unaffected individuals generally have mor...
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| Veröffentlicht in: | PLoS genetics 2013-04, Vol.9 (4), p.e1003415-e1003415 |
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| creator | Krom, Yvonne D Thijssen, Peter E Young, Janet M den Hamer, Bianca Balog, Judit Yao, Zizhen Maves, Lisa Snider, Lauren Knopp, Paul Zammit, Peter S Rijkers, Tonnie van Engelen, Baziel G M Padberg, George W Frants, Rune R Tawil, Rabi Tapscott, Stephen J van der Maarel, Silvère M |
| description | Facioscapulohumeral dystrophy (FSHD) is a progressive muscular dystrophy caused by decreased epigenetic repression of the D4Z4 macrosatellite repeats and ectopic expression of DUX4, a retrogene encoding a germline transcription factor encoded in each repeat. Unaffected individuals generally have more than 10 repeats arrayed in the subtelomeric region of chromosome 4, whereas the most common form of FSHD (FSHD1) is caused by a contraction of the array to fewer than 10 repeats, associated with decreased epigenetic repression and variegated expression of DUX4 in skeletal muscle. We have generated transgenic mice carrying D4Z4 arrays from an FSHD1 allele and from a control allele. These mice recapitulate important epigenetic and DUX4 expression attributes seen in patients and controls, respectively, including high DUX4 expression levels in the germline, (incomplete) epigenetic repression in somatic tissue, and FSHD-specific variegated DUX4 expression in sporadic muscle nuclei associated with D4Z4 chromatin relaxation. In addition we show that DUX4 is able to activate similar functional gene groups in mouse muscle cells as it does in human muscle cells. These transgenic mice therefore represent a valuable animal model for FSHD and will be a useful resource to study the molecular mechanisms underlying FSHD and to test new therapeutic intervention strategies. |
| doi_str_mv | 10.1371/journal.pgen.1003415 |
| format | Article |
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Unaffected individuals generally have more than 10 repeats arrayed in the subtelomeric region of chromosome 4, whereas the most common form of FSHD (FSHD1) is caused by a contraction of the array to fewer than 10 repeats, associated with decreased epigenetic repression and variegated expression of DUX4 in skeletal muscle. We have generated transgenic mice carrying D4Z4 arrays from an FSHD1 allele and from a control allele. These mice recapitulate important epigenetic and DUX4 expression attributes seen in patients and controls, respectively, including high DUX4 expression levels in the germline, (incomplete) epigenetic repression in somatic tissue, and FSHD-specific variegated DUX4 expression in sporadic muscle nuclei associated with D4Z4 chromatin relaxation. In addition we show that DUX4 is able to activate similar functional gene groups in mouse muscle cells as it does in human muscle cells. These transgenic mice therefore represent a valuable animal model for FSHD and will be a useful resource to study the molecular mechanisms underlying FSHD and to test new therapeutic intervention strategies.</description><identifier>ISSN: 1553-7404</identifier><identifier>ISSN: 1553-7390</identifier><identifier>EISSN: 1553-7404</identifier><identifier>DOI: 10.1371/journal.pgen.1003415</identifier><identifier>PMID: 23593020</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Animals ; Binding sites ; Biology ; Cells, Cultured ; Chromatin - genetics ; Chromosomes ; Colleges & universities ; Disease Models, Animal ; DNA Methylation - genetics ; Embryonic Development - genetics ; Epigenesis, Genetic - genetics ; Epigenetic inheritance ; Epigenetics ; Facioscapulohumeral muscular dystrophy ; Gene Expression Regulation, Developmental ; Genes ; Genetic aspects ; Germ Cells - metabolism ; Homeodomain Proteins - genetics ; Homeodomain Proteins - metabolism ; Humans ; Identification and classification ; Mice ; Mice, Transgenic ; Microsatellites (Genetics) ; Muscle, Skeletal - metabolism ; Muscular dystrophy ; Muscular Dystrophy, Facioscapulohumeral - genetics ; Muscular Dystrophy, Facioscapulohumeral - metabolism ; Musculoskeletal system ; Pets ; Proteins ; Repetitive Sequences, Nucleic Acid - genetics ; Rodents ; Testing</subject><ispartof>PLoS genetics, 2013-04, Vol.9 (4), p.e1003415-e1003415</ispartof><rights>COPYRIGHT 2013 Public Library of Science</rights><rights>2013 Krom et al 2013 Krom et al</rights><rights>2013 Krom et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited: Krom YD, Thijssen PE, Young JM, den Hamer B, Balog J, et al. (2013) Intrinsic Epigenetic Regulation of the D4Z4 Macrosatellite Repeat in a Transgenic Mouse Model for FSHD. PLoS Genet 9(4): e1003415. doi:10.1371/journal.pgen.1003415</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c764t-ae1d8075022775e78a5221e15fe10afa70eee946419b62f7b1a6777b32ddf45b3</citedby><cites>FETCH-LOGICAL-c764t-ae1d8075022775e78a5221e15fe10afa70eee946419b62f7b1a6777b32ddf45b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616921/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616921/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,2102,2928,23866,27924,27925,53791,53793,79600,79601</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23593020$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Pearson, Christopher E.</contributor><creatorcontrib>Krom, Yvonne D</creatorcontrib><creatorcontrib>Thijssen, Peter E</creatorcontrib><creatorcontrib>Young, Janet M</creatorcontrib><creatorcontrib>den Hamer, Bianca</creatorcontrib><creatorcontrib>Balog, Judit</creatorcontrib><creatorcontrib>Yao, Zizhen</creatorcontrib><creatorcontrib>Maves, Lisa</creatorcontrib><creatorcontrib>Snider, Lauren</creatorcontrib><creatorcontrib>Knopp, Paul</creatorcontrib><creatorcontrib>Zammit, Peter S</creatorcontrib><creatorcontrib>Rijkers, Tonnie</creatorcontrib><creatorcontrib>van Engelen, Baziel G M</creatorcontrib><creatorcontrib>Padberg, George W</creatorcontrib><creatorcontrib>Frants, Rune R</creatorcontrib><creatorcontrib>Tawil, Rabi</creatorcontrib><creatorcontrib>Tapscott, Stephen J</creatorcontrib><creatorcontrib>van der Maarel, Silvère M</creatorcontrib><title>Intrinsic epigenetic regulation of the D4Z4 macrosatellite repeat in a transgenic mouse model for FSHD</title><title>PLoS genetics</title><addtitle>PLoS Genet</addtitle><description>Facioscapulohumeral dystrophy (FSHD) is a progressive muscular dystrophy caused by decreased epigenetic repression of the D4Z4 macrosatellite repeats and ectopic expression of DUX4, a retrogene encoding a germline transcription factor encoded in each repeat. Unaffected individuals generally have more than 10 repeats arrayed in the subtelomeric region of chromosome 4, whereas the most common form of FSHD (FSHD1) is caused by a contraction of the array to fewer than 10 repeats, associated with decreased epigenetic repression and variegated expression of DUX4 in skeletal muscle. We have generated transgenic mice carrying D4Z4 arrays from an FSHD1 allele and from a control allele. These mice recapitulate important epigenetic and DUX4 expression attributes seen in patients and controls, respectively, including high DUX4 expression levels in the germline, (incomplete) epigenetic repression in somatic tissue, and FSHD-specific variegated DUX4 expression in sporadic muscle nuclei associated with D4Z4 chromatin relaxation. In addition we show that DUX4 is able to activate similar functional gene groups in mouse muscle cells as it does in human muscle cells. These transgenic mice therefore represent a valuable animal model for FSHD and will be a useful resource to study the molecular mechanisms underlying FSHD and to test new therapeutic intervention strategies.</description><subject>Animals</subject><subject>Binding sites</subject><subject>Biology</subject><subject>Cells, Cultured</subject><subject>Chromatin - genetics</subject><subject>Chromosomes</subject><subject>Colleges & universities</subject><subject>Disease Models, Animal</subject><subject>DNA Methylation - genetics</subject><subject>Embryonic Development - genetics</subject><subject>Epigenesis, Genetic - genetics</subject><subject>Epigenetic inheritance</subject><subject>Epigenetics</subject><subject>Facioscapulohumeral muscular dystrophy</subject><subject>Gene Expression Regulation, Developmental</subject><subject>Genes</subject><subject>Genetic aspects</subject><subject>Germ Cells - metabolism</subject><subject>Homeodomain Proteins - genetics</subject><subject>Homeodomain Proteins - metabolism</subject><subject>Humans</subject><subject>Identification and classification</subject><subject>Mice</subject><subject>Mice, Transgenic</subject><subject>Microsatellites (Genetics)</subject><subject>Muscle, Skeletal - metabolism</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Facioscapulohumeral - genetics</subject><subject>Muscular Dystrophy, Facioscapulohumeral - metabolism</subject><subject>Musculoskeletal system</subject><subject>Pets</subject><subject>Proteins</subject><subject>Repetitive Sequences, Nucleic Acid - genetics</subject><subject>Rodents</subject><subject>Testing</subject><issn>1553-7404</issn><issn>1553-7390</issn><issn>1553-7404</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>DOA</sourceid><recordid>eNqVk1-L1DAUxYso7rr6DUQLgujDjPnbtC_Csuu6A4sLrvrgS7htbzsZ2mY2SUW_vakzu0zBByXQhPR3TsK5uUnynJIl5Yq-29jRDdAtty0OS0oIF1Q-SI6plHyhBBEPD9ZHyRPvN5GReaEeJ0eMy4ITRo6TZjUEZwZvqhS3JlphiEuH7dhBMHZIbZOGNabn4rtIe6ic9RCw60zASG0RQmqGFNLgYPBRHsW9HT3Gb41d2liXXtxcnj9NHjXQeXy2n0-SrxcfvpxdLq6uP67OTq8WlcpEWADSOidKEsaUkqhykIxRpLJBSqABRRCxEJmgRZmxRpUUMqVUyVldN0KW_CR5ufPddtbrfUReUy5pQaiSLBKrHVFb2OitMz24X9qC0X82rGs1uJhBhzrnQMuaqpznmRC5BARRY15UTZllspxOe78_bSx7rCuMWUI3M53_Gcxat_aH5hnNCkajwZu9gbO3I_qge-OrGC8MGFOM92a5FBmjMqKvdmgL8WpmaGx0rCZcn_JYS0klEZFa_oWKo8beVHbAxsT9meDtTBCZgD9DC6P3enXz-T_YT__OXn-bs68P2DVCF9beduP0_vwcFDtweobeYXMfNSV6aoq7iuupKfS-KaLsxWGZ7kV3XcB_A4PmBc4</recordid><startdate>20130401</startdate><enddate>20130401</enddate><creator>Krom, Yvonne D</creator><creator>Thijssen, Peter E</creator><creator>Young, Janet M</creator><creator>den Hamer, Bianca</creator><creator>Balog, Judit</creator><creator>Yao, Zizhen</creator><creator>Maves, Lisa</creator><creator>Snider, Lauren</creator><creator>Knopp, Paul</creator><creator>Zammit, Peter S</creator><creator>Rijkers, Tonnie</creator><creator>van Engelen, Baziel G M</creator><creator>Padberg, George W</creator><creator>Frants, Rune R</creator><creator>Tawil, Rabi</creator><creator>Tapscott, Stephen J</creator><creator>van der Maarel, Silvère M</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</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>IOV</scope><scope>ISN</scope><scope>ISR</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20130401</creationdate><title>Intrinsic epigenetic regulation of the D4Z4 macrosatellite repeat in a transgenic mouse model for FSHD</title><author>Krom, Yvonne D ; Thijssen, Peter E ; Young, Janet M ; den Hamer, Bianca ; Balog, Judit ; Yao, Zizhen ; Maves, Lisa ; Snider, Lauren ; Knopp, Paul ; Zammit, Peter S ; Rijkers, Tonnie ; van Engelen, Baziel G M ; Padberg, George W ; Frants, Rune R ; Tawil, Rabi ; Tapscott, Stephen J ; van der Maarel, Silvère M</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c764t-ae1d8075022775e78a5221e15fe10afa70eee946419b62f7b1a6777b32ddf45b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Animals</topic><topic>Binding sites</topic><topic>Biology</topic><topic>Cells, Cultured</topic><topic>Chromatin - genetics</topic><topic>Chromosomes</topic><topic>Colleges & universities</topic><topic>Disease Models, Animal</topic><topic>DNA Methylation - genetics</topic><topic>Embryonic Development - genetics</topic><topic>Epigenesis, Genetic - genetics</topic><topic>Epigenetic inheritance</topic><topic>Epigenetics</topic><topic>Facioscapulohumeral muscular dystrophy</topic><topic>Gene Expression Regulation, Developmental</topic><topic>Genes</topic><topic>Genetic aspects</topic><topic>Germ Cells - metabolism</topic><topic>Homeodomain Proteins - genetics</topic><topic>Homeodomain Proteins - metabolism</topic><topic>Humans</topic><topic>Identification and classification</topic><topic>Mice</topic><topic>Mice, Transgenic</topic><topic>Microsatellites (Genetics)</topic><topic>Muscle, Skeletal - metabolism</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Facioscapulohumeral - genetics</topic><topic>Muscular Dystrophy, Facioscapulohumeral - metabolism</topic><topic>Musculoskeletal system</topic><topic>Pets</topic><topic>Proteins</topic><topic>Repetitive Sequences, Nucleic Acid - genetics</topic><topic>Rodents</topic><topic>Testing</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Krom, Yvonne D</creatorcontrib><creatorcontrib>Thijssen, Peter E</creatorcontrib><creatorcontrib>Young, Janet M</creatorcontrib><creatorcontrib>den Hamer, Bianca</creatorcontrib><creatorcontrib>Balog, Judit</creatorcontrib><creatorcontrib>Yao, Zizhen</creatorcontrib><creatorcontrib>Maves, Lisa</creatorcontrib><creatorcontrib>Snider, Lauren</creatorcontrib><creatorcontrib>Knopp, Paul</creatorcontrib><creatorcontrib>Zammit, Peter S</creatorcontrib><creatorcontrib>Rijkers, Tonnie</creatorcontrib><creatorcontrib>van Engelen, Baziel G M</creatorcontrib><creatorcontrib>Padberg, George W</creatorcontrib><creatorcontrib>Frants, Rune R</creatorcontrib><creatorcontrib>Tawil, Rabi</creatorcontrib><creatorcontrib>Tapscott, Stephen J</creatorcontrib><creatorcontrib>van der Maarel, Silvère M</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: Opposing Viewpoints</collection><collection>Gale In Context: Canada</collection><collection>Gale In Context: Science</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PLoS genetics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Krom, Yvonne D</au><au>Thijssen, Peter E</au><au>Young, Janet M</au><au>den Hamer, Bianca</au><au>Balog, Judit</au><au>Yao, Zizhen</au><au>Maves, Lisa</au><au>Snider, Lauren</au><au>Knopp, Paul</au><au>Zammit, Peter S</au><au>Rijkers, Tonnie</au><au>van Engelen, Baziel G M</au><au>Padberg, George W</au><au>Frants, Rune R</au><au>Tawil, Rabi</au><au>Tapscott, Stephen J</au><au>van der Maarel, Silvère M</au><au>Pearson, Christopher E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Intrinsic epigenetic regulation of the D4Z4 macrosatellite repeat in a transgenic mouse model for FSHD</atitle><jtitle>PLoS genetics</jtitle><addtitle>PLoS Genet</addtitle><date>2013-04-01</date><risdate>2013</risdate><volume>9</volume><issue>4</issue><spage>e1003415</spage><epage>e1003415</epage><pages>e1003415-e1003415</pages><issn>1553-7404</issn><issn>1553-7390</issn><eissn>1553-7404</eissn><abstract>Facioscapulohumeral dystrophy (FSHD) is a progressive muscular dystrophy caused by decreased epigenetic repression of the D4Z4 macrosatellite repeats and ectopic expression of DUX4, a retrogene encoding a germline transcription factor encoded in each repeat. Unaffected individuals generally have more than 10 repeats arrayed in the subtelomeric region of chromosome 4, whereas the most common form of FSHD (FSHD1) is caused by a contraction of the array to fewer than 10 repeats, associated with decreased epigenetic repression and variegated expression of DUX4 in skeletal muscle. We have generated transgenic mice carrying D4Z4 arrays from an FSHD1 allele and from a control allele. These mice recapitulate important epigenetic and DUX4 expression attributes seen in patients and controls, respectively, including high DUX4 expression levels in the germline, (incomplete) epigenetic repression in somatic tissue, and FSHD-specific variegated DUX4 expression in sporadic muscle nuclei associated with D4Z4 chromatin relaxation. In addition we show that DUX4 is able to activate similar functional gene groups in mouse muscle cells as it does in human muscle cells. These transgenic mice therefore represent a valuable animal model for FSHD and will be a useful resource to study the molecular mechanisms underlying FSHD and to test new therapeutic intervention strategies.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>23593020</pmid><doi>10.1371/journal.pgen.1003415</doi><oa>free_for_read</oa></addata></record> |
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| subjects | Animals Binding sites Biology Cells, Cultured Chromatin - genetics Chromosomes Colleges & universities Disease Models, Animal DNA Methylation - genetics Embryonic Development - genetics Epigenesis, Genetic - genetics Epigenetic inheritance Epigenetics Facioscapulohumeral muscular dystrophy Gene Expression Regulation, Developmental Genes Genetic aspects Germ Cells - metabolism Homeodomain Proteins - genetics Homeodomain Proteins - metabolism Humans Identification and classification Mice Mice, Transgenic Microsatellites (Genetics) Muscle, Skeletal - metabolism Muscular dystrophy Muscular Dystrophy, Facioscapulohumeral - genetics Muscular Dystrophy, Facioscapulohumeral - metabolism Musculoskeletal system Pets Proteins Repetitive Sequences, Nucleic Acid - genetics Rodents Testing |
| title | Intrinsic epigenetic regulation of the D4Z4 macrosatellite repeat in a transgenic mouse model for FSHD |
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