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Series GSE29775 Query DataSets for GSE29775
Status Public on Aug 01, 2011
Title Rhabdomyosarcomas in aging A/J mice
Organism Mus musculus
Experiment type Expression profiling by array
Summary Rhabdomyosarcomas (RSCs) are skeletal muscle neoplasms found in humans and domestic mammals. The A/J inbred strain developed a high frequency of RSC by 20 months of age while BALB/cJ and BALB/cByJ also develop RSC but less frequently. These neoplasms affected 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 that invaded locally. 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 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.
Overall design Total RNA obtained from skeletal muscle samples from neoplastic and non-neoplastic tissues. Four rhabdomyosarcomas (~450 day old male A/J mice) and 8 nonneoplastic skeletal muscle samples (425-550 day old male A/J mice) were used
Contributor(s) Sher RB, Cox GA, Mills KD, Sundberg JP
Citation(s) 21853140
Submission date Jun 07, 2011
Last update date Mar 23, 2012
Contact name Roger Sher
Phone 207-288-6503
Fax 207-288-6077
Organization name The Jackson Laboratory
Street address 600 Main Street
City Bar Harbor
ZIP/Postal code 04609
Country USA
Platforms (1)
GPL6481 Illumina mouse-6 v1.1 expression beadchip [Array_Address_Id version]
Samples (12)
GSM738155 muscle_rhabdo_rep1
GSM738156 muscle_control_rep1
GSM738157 muscle_rhabdo_rep2
BioProject PRJNA141047

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Supplementary file Size Download File type/resource
GSE29775_non-normalized.txt.gz 3.9 Mb (ftp)(http) TXT
Processed data included within Sample table

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