Hdl Handle:
http://hdl.handle.net/10675.2/668
Title:
Rhabdomyosarcomas in Aging A/J Mice
Authors:
Sher, Roger B.; Cox, Gregory A. ( 0000-0003-3681-8296 ) ; Mills, Kevin D.; Sundberg, John P.
Abstract:
Rhabdomyosarcomas (RSCs) are skeletal muscle neoplasms found in humans and domestic mammals. The A/J inbred strain developed a high frequency (between 70â 80%) of adult pleomorphic type (APT) RSC at >20 months of age while BALB/cByJ also develop RSC but less frequently. These neoplasms invaded skeletal muscle surrounding either the axial or proximal appendicular skeleton and were characterized by pleomorphic cells with abundant eosinophilic cytoplasm, multiple nuclei, and cross striations. The diagnosis was confirmed by detection of alpha-sarcomeric actin and myogenin in the neoplastic cells using immunocytochemistry. The A/J strain, but not the related BALB/c substrains, is also characterised by a progressive muscular dystrophy homologous to limb-girdle muscular dystrophy type 2B. The association between the development of RSC in similar muscle groups to those most severely affected by the progressive muscular dystrophy suggested that these neoplasms developed from abnormal regeneration of the skeletal muscle exacerbated by the dysferlin mutation. Transcriptome analyses of RSCs revealed marked downregulation of genes in muscular development and function signaling networks. Non-synonymous coding SNPs were found in Myl1, Abra, Sgca, Ttn, and Kcnj12 suggesting these may be important in the pathogenesis of RSC. These studies suggest that A strains of mice can be useful models for dissecting the molecular genetic basis for development, progression, and ultimately for testing novel anticancer therapeutic agents dealing with rhabdomyosarcoma.
Editors:
McNeil, Paul L.
Citation:
PLoS One. 2011 Aug 10; 6(8):e23498
Issue Date:
10-Aug-2011
URI:
http://hdl.handle.net/10675.2/668
DOI:
10.1371/journal.pone.0023498
PubMed ID:
21853140
PubMed Central ID:
PMC3154500
Type:
Article
ISSN:
1932-6203
Appears in Collections:
Department of Cellular Biology and Anatomy Faculty Papers

Full metadata record

DC FieldValue Language
dc.contributor.authorSher, Roger B.en_US
dc.contributor.authorCox, Gregory A.en_US
dc.contributor.authorMills, Kevin D.en_US
dc.contributor.authorSundberg, John P.en_US
dc.contributor.editorMcNeil, Paul L.-
dc.date.accessioned2012-10-26T16:29:27Z-
dc.date.available2012-10-26T16:29:27Z-
dc.date.issued2011-08-10en_US
dc.identifier.citationPLoS One. 2011 Aug 10; 6(8):e23498en_US
dc.identifier.issn1932-6203en_US
dc.identifier.pmid21853140en_US
dc.identifier.doi10.1371/journal.pone.0023498en_US
dc.identifier.urihttp://hdl.handle.net/10675.2/668-
dc.description.abstractRhabdomyosarcomas (RSCs) are skeletal muscle neoplasms found in humans and domestic mammals. The A/J inbred strain developed a high frequency (between 70â 80%) of adult pleomorphic type (APT) RSC at >20 months of age while BALB/cByJ also develop RSC but less frequently. These neoplasms invaded skeletal muscle surrounding either the axial or proximal appendicular skeleton and were characterized by pleomorphic cells with abundant eosinophilic cytoplasm, multiple nuclei, and cross striations. The diagnosis was confirmed by detection of alpha-sarcomeric actin and myogenin in the neoplastic cells using immunocytochemistry. The A/J strain, but not the related BALB/c substrains, is also characterised by a progressive muscular dystrophy homologous to limb-girdle muscular dystrophy type 2B. The association between the development of RSC in similar muscle groups to those most severely affected by the progressive muscular dystrophy suggested that these neoplasms developed from abnormal regeneration of the skeletal muscle exacerbated by the dysferlin mutation. Transcriptome analyses of RSCs revealed marked downregulation of genes in muscular development and function signaling networks. Non-synonymous coding SNPs were found in Myl1, Abra, Sgca, Ttn, and Kcnj12 suggesting these may be important in the pathogenesis of RSC. These studies suggest that A strains of mice can be useful models for dissecting the molecular genetic basis for development, progression, and ultimately for testing novel anticancer therapeutic agents dealing with rhabdomyosarcoma.en_US
dc.rightsSher 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.en_US
dc.subjectResearch Articleen_US
dc.subjectBiologyen_US
dc.subjectBiochemistryen_US
dc.subjectCytochemistryen_US
dc.subjectImmunocytochemistryen_US
dc.subjectBiophysicsen_US
dc.subjectCell Motilityen_US
dc.subjectActin Filamentsen_US
dc.subjectGeneticsen_US
dc.subjectCancer Geneticsen_US
dc.subjectGenetic Mutationen_US
dc.subjectGenomicsen_US
dc.subjectHistologyen_US
dc.subjectModel Organismsen_US
dc.subjectAnimal Modelsen_US
dc.subjectMouseen_US
dc.subjectMolecular Cell Biologyen_US
dc.subjectCellular Typesen_US
dc.subjectMuscle Cellsen_US
dc.subjectSystems Biologyen_US
dc.subjectMedicineen_US
dc.subjectOncologyen_US
dc.subjectCancer Risk Factorsen_US
dc.subjectAging and Canceren_US
dc.subjectGenetic Causes of Canceren_US
dc.subjectCancer Detection and Diagnosisen_US
dc.subjectCancers and Neoplasmsen_US
dc.subjectPhysicsen_US
dc.subjectBiophysicsen_US
dc.subjectCell Motilityen_US
dc.subjectActin Filamentsen_US
dc.subjectVeterinary Scienceen_US
dc.subjectVeterinary Diseasesen_US
dc.subjectVeterinary Informaticsen_US
dc.subjectVeterinary Pathologyen_US
dc.titleRhabdomyosarcomas in Aging A/J Miceen_US
dc.typeArticleen_US
dc.identifier.pmcidPMC3154500en_US
dc.contributor.corporatenameDepartment of Cellular Biology and Anatomy-
dc.contributor.corporatenameCollege of Graduate Studies-

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