Expression of Connective Tissue Growth Factor (CTGF/CCN2) in a Mouse Model of Rhabdomyosarcomagenesis

Stefania CROCI1, Lorena LANDUZZI2, Giordano NICOLETTI2, Arianna PALLADINI1, Agnese ANTOGNOLI1, Carla DE GIOVANNI1, Patrizia NANNI1, Pier-Luigi LOLLINI1

1Cancer Research Section, Department of Experimental Pathology, University of Bologna, Bologna, Italy
2Laboratorio di Ricerca Oncologica, Istituti Ortopedici Rizzoli, Bologna, Italy


Connective tissue growth factor (CTGF/CCN2) is a cysteine-rich matricellular protein that belongs to the CCN (CYR61, CTGF, NOV) protein family. It is highly expressed by human rhabdomyosarcoma cells and sustains their survival. In this study we investigated CCN2 expression in a mouse model of spontaneous rhabdomyosarcomagenesis that combines HER-2/neu oncogene activation and p53 oncosuppressor gene inactivation (BALB-p53neu mice). Murine rhabdomyosarcoma cells showed a 4-26 fold increase in CCN2 mRNA expression regarding to normal thigh muscle. Moreover, they expressed CCN2 protein at levels comparable to human rhabdomyosarcoma cells. Therefore BALBp53neu mice might be useful for the evaluation of the role played by CCN2 in rhabdomyosarcoma in vivo. Pathology & Oncology Research, Vol 13, Nr 4, 336-339, 2007

Key words: rhabdomyosarcoma; mouse model; CCN proteins; CCN2/CTGF

Received: Dec 4, 2006; accepted: Nov 10, 2007
Correspondence: Pier-Luigi LOLLINI, Cancer Research Section, Department of Experimental Pathology, University of Bologna, Viale Filopanti 22 Bologna I-40126, Italy; Tel: +39-051-2094786, Fax: +39-051-242169; E-mail:

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