title: Common fragile site tumor suppressor genes and corresponding mouse models of cancer.
abstract: Chromosomal common fragile sites (CFSs) are specific mammalian genomic regions that show an increased frequency of gaps and breaks when cells are exposed to replication stress in vitro. CFSs are also consistently involved in chromosomal abnormalities in vivo related to cancer. Interestingly, several CFSs contain one or more tumor suppressor genes whose structure and function are often affected by chromosomal fragility. The two most active fragile sites in the human genome are FRA3B and FRA16D where the tumor suppressor genes FHIT and WWOX are located, respectively. The best approach to study tumorigenic effects of altered tumor suppressors located at CFSs in vivo is to generate mouse models in which these genes are inactivated. This paper summarizes our present knowledge on mouse models of cancer generated by knocking out tumor suppressors of CFS.
authors.affiliation: Department of Molecular Virology, Immunology, and Medical Genetics, The Ohio State University, Columbus, OH 43210, USA.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Drusco
authors.forename: Yuri
authors.initials: Y
authors.lastname: Pekarsky
authors.forename: Stefan
authors.initials: S
authors.lastname: Costinean
authors.forename: Anna
authors.initials: A
authors.lastname: Antenucci
authors.forename: Laura
authors.initials: L
authors.lastname: Conti
authors.forename: Stefano
authors.initials: S
authors.lastname: Volinia
authors.forename: Rami I
authors.initials: RI
authors.lastname: Aqeilan
authors.forename: Kay
authors.initials: K
authors.lastname: Huebner
authors.forename: Nicola
authors.initials: N
authors.lastname: Zanesi
mesh_terms: D017766:Acid Anhydride Hydrolases / Q000235:genetics; D000818:Animals; D043283:Chromosome Fragile Sites / Q000235:genetics*; D004195:Disease Models, Animal; D016147:Genes, Tumor Suppressor; D006801:Humans; D051379:Mice; D009363:Neoplasm Proteins / Q000235:genetics; D009369:Neoplasms / Q000235:genetics*; D010088:Oxidoreductases / Q000235:genetics; D000074584:WW Domain-Containing Oxidoreductase; D000099170:Bis(5'-adenosyl)triphosphatase
chemical_list: D017766:Acid Anhydride Hydrolases; D009363:Neoplasm Proteins; D010088:Oxidoreductases; D000074584:WW Domain-Containing Oxidoreductase; D000099170:Bis(5'-adenosyl)triphosphatase; C428130:Wwox protein, mouse