IRIZIO : a novel gene cooperating with PAX3-FOXO1 in alveolar rhabdomyosarcoma (ARMS)
- 22 December 2010
- journal article
- Published by Oxford University Press (OUP) in Carcinogenesis: Integrative Cancer Research
- Vol. 32 (4), 452-461
- https://doi.org/10.1093/carcin/bgq273
Abstract
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children with an annual incidence of five new cases per million. Alveolar rhabdomyosarcoma (ARMS) is characterized by the t(2;13) or t(1;13) chromosomal translocations, which generate the PAX3-FOXO1 or PAX7-FOXO1 fusion genes, respectively. The oncogenic activity of PAX3-FOXO1 has been demonstrated in vitro and in vivo, yet expression of the fusion protein alone in primary myoblasts or a mouse model is insufficient for tumorigenic transformation. To identify genes cooperating with PAX3-FOXO1 in ARMS tumorigenesis, we generated a retroviral complementary DNA (cDNA) expression library from the Rh30 ARMS cell line. Arf-/- myoblasts expressing PAX3-FOXO1 and the retroviral cDNA library rapidly formed tumors after subcutaneous injection into NOD-SCID mice. Tumors formed by Arf-/-/PAX3-FOXO1/MarX-library myoblasts contained an unknown cDNA, encoding the C-terminus of the Homo sapiens hypothetical protein, FLJ10404, herein named IRIZIO. Expression of full length IRIZIO cDNA also cooperated with PAX3-FOXO1 in the transformation of Arf-/- myoblasts. Given that IRIZIO is expressed at increased levels in RMS, it might contribute to rhabdomyosarcomagenesis in humans.Keywords
This publication has 25 references indexed in Scilit:
- Alternative splicing and diseaseBiochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2009
- Altered expression and molecular abnormalities of cell-cycle-regulatory proteins in rhabdomyosarcomaLaboratory Investigation, 2004
- FKHR (FOXO1a) is required for myotube fusion of primary mouse myoblastsThe EMBO Journal, 2003
- Pax3-FKHR Knock-In Mice Show Developmental Aberrations but Do Not Develop TumorsMolecular and Cellular Biology, 2002
- The HIV-1 Rev Activation Domain is a nuclear export signal that accesses an export pathway used by specific cellular RNAsCell, 1995
- Pax3 Inhibits Myogenic Differentiation of Cultured Myoblast CellsPublished by Elsevier BV ,1995
- The PAX3-FKHR Fusion Protein Created by the t(2;13) Translocation in Alveolar Rhabdomyosarcomas Is A More Potent Transcriptional Activator than PAX3Molecular and Cellular Biology, 1995
- Frequency and diversity of p53 mutations in childhood rhabdomyosarcoma.1992
- The nucleoplasmin nuclear location sequence is larger and more complex than that of SV-40 large T antigen.The Journal of cell biology, 1988
- A specific chromosomal abnormality in rhabdomyosarcomaCytogenetic and Genome Research, 1987