Rhabdomyosarcomas in Aging A/J Mice

摘要: 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 age while BALB/cByJ also develop but less frequently. These invaded surrounding either the axial or proximal appendicular skeleton were characterized by cells with abundant eosinophilic cytoplasm, multiple nuclei, cross striations. diagnosis was confirmed detection alpha-sarcomeric actin myogenin neoplastic using immunocytochemistry. strain, not related BALB/c substrains, is characterised progressive muscular dystrophy homologous to limb-girdle 2B. association between development similar groups those most severely affected suggested that these from abnormal regeneration exacerbated dysferlin mutation. Transcriptome analyses RSCs revealed marked downregulation genes function signaling networks. Non-synonymous coding SNPs Myl1, Abra, Sgca, Ttn, Kcnj12 suggesting may be important pathogenesis RSC. studies suggest A strains mice can useful models for dissecting molecular genetic basis development, progression, ultimately testing novel anticancer therapeutic agents dealing rhabdomyosarcoma.