Academic Journals Database
Disseminating quality controlled scientific knowledge

Alveolar rhabdomyosarcoma – The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis

Author(s): Marshall Amy D | Grosveld Gerard C

Journal: Skeletal Muscle
ISSN 2044-5040

Volume: 2;
Issue: 1;
Start page: 25;
Date: 2012;
Original page

Keywords: Alveolar rhabdomyosarcoma | PAX3-FOXO1 | PAX7-FOXO1 | FGFR4 | CNR1 | IRIZIO | N-MYC | IGF2 | MET | CXCR4 | p53 | MDM2 | P-Cadherin | TFAP2B | miR17-92

Abstract Rhabdomyosarcoma is a soft tissue sarcoma arising from cells of a mesenchymal or skeletal muscle lineage. Alveolar rhabdomyosarcoma (ARMS) is more aggressive than the more common embryonal (ERMS) subtype. ARMS is more prone to metastasis and carries a poorer prognosis. In contrast to ERMS, the majority of ARMS tumors carry one of several characteristic chromosomal translocations, such as t(2;13)(q35;q14), which results in the expression of a PAX3-FOXO1 fusion transcription factor. In this review we discuss the genes that cooperate with PAX3-FOXO1, as well as the target genes of the fusion transcription factor that contribute to various aspects of ARMS tumorigenesis. The characterization of these pathways will lead to a better understanding of ARMS tumorigenesis and will allow the design of novel targeted therapies that will lead to better treatment for this aggressive pediatric tumor.
Affiliate Program      Why do you need a reservation system?