Journal Article

<i>IRIZIO</i>: a novel gene cooperating with PAX3-FOXO1 in alveolar rhabdomyosarcoma (ARMS)

Fabrizio Picchione, Colin Pritchard, Irina Lagutina, Laura Janke and Gerard C. Grosveld

in Carcinogenesis

Volume 32, issue 4, pages 452-461
Published in print April 2011 | ISSN: 0143-3334
Published online December 2010 | e-ISSN: 1460-2180 | DOI:
IRIZIO: a novel gene cooperating with PAX3-FOXO1 in alveolar rhabdomyosarcoma (ARMS)

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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.

Journal Article.  6968 words.  Illustrated.

Subjects: Clinical Cytogenetics and Molecular Genetics

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