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GOK: A Gene at 11p15 Involved in Rhabdomyosarcoma and Rhabdoid Tumor Development¹

Department of Experimental and Diagnostic Medicine, Section of Microbiology, and Interdepartment Center for Biotechnology, University of Ferrara, Ferrara, 44100 Italy [S. S., G. B-B., M. N.], and Kimmel Cancer Institute and Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, Pennsylvania 19107 [C. M. C.]

The expression of GOK, a gene recently identified at 11p15.5, was studied in breast cancer, rhabdomyosarcoma, and rhabdoid tumor cell lines. In these neoplasms, deletions at 11p15 and suppression of tumorigenicity induced by a normal human chromosome 11 were previously demonstrated. Whereas breast cancer cell lines express readily detectable levels of GOK mRNA, expression is absent in rhabdomyosarcoma and rhabdoid tumor cell lines. This is in contrast with the high expression of GOK in skeletal muscle, the normal tissue of origin of rhabdomyosarcomas, suggesting that down-regulation of GOK expression could be involved in tumor development. In agreement with this hypothesis, transfection of GOK cDNA into G401 derived from a rhabdoid tumor and RD cells derived from a rhabdomyosarcoma that do not express detectable levels of GOK mRNA, induced cell death. Because GOK expression is not compatible with growth of these tumor cells, these results support the hypothesis that loss of GOK expression plays a role in tumor establishment or progression and suggest that GOK may act as a recessive tumor suppressor gene in rhabdomyosarcomas and rhabdoid tumors. On the contrary, transfection of GOK cDNA into the breast cancer cell line HBL100 produced no detectable effects, indicating that the growth-suppressive effect of GOK in RD and G401 cells was specific. Because rhabdomyosarcomas have been observed in cases of Beckwith-Wiedemann syndrome, a genetic disorder linked to 11p15, a role of GOK in this disease cannot be excluded.

¹ Supported by Telethon Grant E.366, Associazione Italiana per la Ricerca sul Cancro, Consiglio Nazionale delle Ricerche, Progetto Finalizzato "Applicazioni Cliniche della Ricerca Oncologica" and by National Cancer Institute Grant CA56366.

² To whom requests for reprints should be addressed, at Dipartimento di Medicina Sperimentale e Diagnostica, Sezione di Microbiologia, Università di Ferrara, via Luigi Borsari, 46, Ferrara, 44100 Italy. Phone: 39-532-291395; Fax: 39-532-247618; E-mail: ngm@ifeuniv.unife.it.

Received 7/24/97. Accepted 8/29/97.

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