Wnt/Wingless Pathway Activation and Chromosome 6 Loss Characterise a Distinct Molecular Sub-Group of Medulloblastomas Associated with a Favourable Prognosis
The accurate assessment of disease risk remains a major goal in children with medulloblastoma. Activation of the canonical Wnt/Wingless (Wnt/Wg) signalling pathway occurs in up to 25% of cases and is associated with a favourable disease outcome. To explore the molecular pathogenesis of Wnt/Wg-active...
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| Veröffentlicht in: | Cell cycle (Georgetown, Tex.) Tex.), 2006-11, Vol.5 (22), p.2666-2670 |
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| container_issue | 22 |
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| container_title | Cell cycle (Georgetown, Tex.) |
| container_volume | 5 |
| creator | Clifford, Steven C. Lusher, Meryl E. Lindsey, Janet C. Langdon, Jacueline A. Gilbertson, Richard J. Straughton, Debbie Ellison, David W. |
| description | The accurate assessment of disease risk remains a major goal in children with medulloblastoma. Activation of the canonical Wnt/Wingless (Wnt/Wg) signalling pathway occurs in up to 25% of cases and is associated with a favourable disease outcome. To explore the molecular pathogenesis of Wnt/Wg-active medulloblastomas, and to investigate any genetic basis for their observed clinical behaviour, we assessed a series of primary medulloblastomas for evidence of Wnt/Wg pathway activation, alongside a genome-wide analysis of associated copy-number aberrations. Cases displaying evidence of Wnt/Wg activation (CTNNB1 mutation and/or β-catenin nuclear stabilisation) were exclusively associated with a distinct genomic signature involving loss of an entire copy of chromosome 6 but few other aberrations (p |
| doi_str_mv | 10.4161/cc.5.22.3446 |
| format | Article |
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Activation of the canonical Wnt/Wingless (Wnt/Wg) signalling pathway occurs in up to 25% of cases and is associated with a favourable disease outcome. To explore the molecular pathogenesis of Wnt/Wg-active medulloblastomas, and to investigate any genetic basis for their observed clinical behaviour, we assessed a series of primary medulloblastomas for evidence of Wnt/Wg pathway activation, alongside a genome-wide analysis of associated copy-number aberrations. Cases displaying evidence of Wnt/Wg activation (CTNNB1 mutation and/or β-catenin nuclear stabilisation) were exclusively associated with a distinct genomic signature involving loss of an entire copy of chromosome 6 but few other aberrations (p</description><identifier>ISSN: 1538-4101</identifier><identifier>EISSN: 1551-4005</identifier><identifier>DOI: 10.4161/cc.5.22.3446</identifier><identifier>PMID: 17172831</identifier><language>eng</language><publisher>United States: Taylor & Francis</publisher><subject>Adolescent ; beta Catenin - genetics ; beta Catenin - metabolism ; Binding ; Biology ; Bioscience ; Calcium ; Cancer ; Cell ; Cerebellar Neoplasms - classification ; Cerebellar Neoplasms - diagnosis ; Cerebellar Neoplasms - genetics ; Child ; Child, Preschool ; Chromosome Deletion ; Chromosomes, Human, Pair 6 ; Cohort Studies ; Cycle ; Female ; Humans ; Infant ; Landes ; Male ; Medulloblastoma - classification ; Medulloblastoma - diagnosis ; Medulloblastoma - genetics ; Organogenesis ; Prognosis ; Proteins ; Signal Transduction ; Wnt Proteins - genetics ; Wnt Proteins - metabolism</subject><ispartof>Cell cycle (Georgetown, Tex.), 2006-11, Vol.5 (22), p.2666-2670</ispartof><rights>Copyright © 2006 Landes Bioscience 2006</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c448t-c3b55759abdaaac627342bf711fc6cdd06df4ac84928541f2652f84954fafd793</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17172831$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Clifford, Steven C.</creatorcontrib><creatorcontrib>Lusher, Meryl E.</creatorcontrib><creatorcontrib>Lindsey, Janet C.</creatorcontrib><creatorcontrib>Langdon, Jacueline A.</creatorcontrib><creatorcontrib>Gilbertson, Richard J.</creatorcontrib><creatorcontrib>Straughton, Debbie</creatorcontrib><creatorcontrib>Ellison, David W.</creatorcontrib><title>Wnt/Wingless Pathway Activation and Chromosome 6 Loss Characterise a Distinct Molecular Sub-Group of Medulloblastomas Associated with a Favourable Prognosis</title><title>Cell cycle (Georgetown, Tex.)</title><addtitle>Cell Cycle</addtitle><description>The accurate assessment of disease risk remains a major goal in children with medulloblastoma. Activation of the canonical Wnt/Wingless (Wnt/Wg) signalling pathway occurs in up to 25% of cases and is associated with a favourable disease outcome. To explore the molecular pathogenesis of Wnt/Wg-active medulloblastomas, and to investigate any genetic basis for their observed clinical behaviour, we assessed a series of primary medulloblastomas for evidence of Wnt/Wg pathway activation, alongside a genome-wide analysis of associated copy-number aberrations. Cases displaying evidence of Wnt/Wg activation (CTNNB1 mutation and/or β-catenin nuclear stabilisation) were exclusively associated with a distinct genomic signature involving loss of an entire copy of chromosome 6 but few other aberrations (p</description><subject>Adolescent</subject><subject>beta Catenin - genetics</subject><subject>beta Catenin - metabolism</subject><subject>Binding</subject><subject>Biology</subject><subject>Bioscience</subject><subject>Calcium</subject><subject>Cancer</subject><subject>Cell</subject><subject>Cerebellar Neoplasms - classification</subject><subject>Cerebellar Neoplasms - diagnosis</subject><subject>Cerebellar Neoplasms - genetics</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Chromosome Deletion</subject><subject>Chromosomes, Human, Pair 6</subject><subject>Cohort Studies</subject><subject>Cycle</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Landes</subject><subject>Male</subject><subject>Medulloblastoma - classification</subject><subject>Medulloblastoma - diagnosis</subject><subject>Medulloblastoma - genetics</subject><subject>Organogenesis</subject><subject>Prognosis</subject><subject>Proteins</subject><subject>Signal Transduction</subject><subject>Wnt Proteins - genetics</subject><subject>Wnt Proteins - metabolism</subject><issn>1538-4101</issn><issn>1551-4005</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNptkU1vEzEQQFcIREvhxhn5xIlN1l57P45RSgtSKioB6tGaHduNkdcOtrdR_gs_lo0S4MJpZqQ3b0YzRfGWVgtOG7pEXIgFY4ua8-ZZcUmFoCWvKvH8mNddyWlFL4pXKf2oKta1PX1ZXNCWtqyr6WXx68Hn5YP1j06nRO4hb_dwICvM9gmyDZ6AV2S9jWEMKYyaNGQTZnC9hQiYdbRJEyDXNmXrMZO74DRODiL5Og3lbQzTjgRD7rSanAuDg5TDCImsUgpoIWtF9jZvZ8UNPIUpwuA0uY_h0Ydk0-vihQGX9JtzvCq-33z8tv5Ubr7cfl6vNiVy3uUS60GIVvQwKADAhrU1Z4NpKTXYoFJVowwH7HjPOsGpYY1gZq4EN2BU29dXxfuTdxfDz0mnLEebUDsHXocpyaZjDe9pNYMfTiDG-QpRG7mLdoR4kLSSx29IRCkkY_L4jRl_d_ZOw6jVP_h8_hlYnoB5ktJpsCGh1R71X3T2QcwWnf6jbE8d1psQR9iH6JTMcHAhmggebZL1f5f5DXG-rOk</recordid><startdate>20061115</startdate><enddate>20061115</enddate><creator>Clifford, Steven C.</creator><creator>Lusher, Meryl E.</creator><creator>Lindsey, Janet C.</creator><creator>Langdon, Jacueline A.</creator><creator>Gilbertson, Richard J.</creator><creator>Straughton, Debbie</creator><creator>Ellison, David W.</creator><general>Taylor & Francis</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></search><sort><creationdate>20061115</creationdate><title>Wnt/Wingless Pathway Activation and Chromosome 6 Loss Characterise a Distinct Molecular Sub-Group of Medulloblastomas Associated with a Favourable Prognosis</title><author>Clifford, Steven C. ; 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| ispartof | Cell cycle (Georgetown, Tex.), 2006-11, Vol.5 (22), p.2666-2670 |
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| source | MEDLINE; EZB Electronic Journals Library |
| subjects | Adolescent beta Catenin - genetics beta Catenin - metabolism Binding Biology Bioscience Calcium Cancer Cell Cerebellar Neoplasms - classification Cerebellar Neoplasms - diagnosis Cerebellar Neoplasms - genetics Child Child, Preschool Chromosome Deletion Chromosomes, Human, Pair 6 Cohort Studies Cycle Female Humans Infant Landes Male Medulloblastoma - classification Medulloblastoma - diagnosis Medulloblastoma - genetics Organogenesis Prognosis Proteins Signal Transduction Wnt Proteins - genetics Wnt Proteins - metabolism |
| title | Wnt/Wingless Pathway Activation and Chromosome 6 Loss Characterise a Distinct Molecular Sub-Group of Medulloblastomas Associated with a Favourable Prognosis |
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