Reference SummarySher RB, PLoS One 2011;6(8):e23498

Title

Rhabdomyosarcomas in aging A/J mice.

Authors

Sher RB; Cox GA; Mills KD; Sundberg JP

Journal

PLoS One

Volume

6

Issue

8

Year

2011

Pages

e23498

Abstract

Rhabdomyosarcomas (RSCs) are skeletal muscle neoplasms found in humans and domestic mammals. The A/J inbred strain developed a high frequency (between 70-80%) 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.

Links

21853140 – National Library of Medicine/PubMed

Models

Strain Model Name Treatment Agent(s) Organ Affected Frequency Model Details
A/J Muscle - Striated - Skeletal lesion Muscle - Striated - Skeletal

observed

A/J Muscle - Striated - Skeletal neoplasm Muscle - Striated - Skeletal

observed

A/J Muscle - Striated - Skeletal normal tissue (control) Muscle - Striated - Skeletal

not applicable

A/J Muscle - Striated - Skeletal rhabdomyosarcoma Muscle - Striated - Skeletal

observed

A/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0 - 78.95

BALB/cJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

1.05

BALB/cByJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0 - 11.11

129S1/SvImJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

AKR/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

BTBR T+ Itpr3tf/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

BUB/BnJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C3H/HeJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C57BL/6J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C57BL/10J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C57BLKS/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C57BR/cdJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

C57L/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

CBA/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

DBA/2J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

FVB/NJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

KK/HlJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

LP/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

MRL/MpJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

NON/ShiLtJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

NZO/HlLtJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

NZW/LacJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

P/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

PL/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

PWD/PhJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

RIIIS/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

SJL/J-Dysfim Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

SM/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

SWR/J Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

WSB/EiJ Muscle - Striated - Skeletal rhabdomyosarcoma - pleomorphic Muscle - Striated - Skeletal

0

BALB/cByJ Muscle - Striated - Skeletal rhabdomyosarcoma - well differentiated Muscle - Striated - Skeletal

observed

A/J Muscle - Striated - Skeletal tumor Muscle - Striated - Skeletal

observed

SJL/J-Dysfim (Unspecified organ) reticulum cell sarcoma (Unspecified organ)

observed

SJL/J-Dysfim (Unspecified organ) tumor (Unspecified organ)

observed