S. Venkatachalam et La. Donehower, MURINE TUMOR-SUPPRESSOR MODELS, Mutation research. Fundamental and molecular mechanisms of mutagenesis, 400(1-2), 1998, pp. 391-407
Tumor suppressor genes have been shown to be necessary for proper main
tenance of cell growth control. Inactivation of these genes in the ger
mline of humans is linked to inherited cancer predisposition. Moreover
, sporadically arising human tumors often have somatic mutations in tu
mor suppressor genes. During the past few years, advances in molecular
and cellular biology have led to the creation of animal models that h
ave germline mutations of various tumor suppressor genes. Such mice po
tentially represent important animal models for familial cancer predis
position syndromes, and the study of the tumorigenesis process has bee
n greatly assisted by their development. Such models have also demonst
rated the importance of tumor suppressor function in embryonic develop
ment. In this review, we describe mice with inactivated germline tumor
suppressor genes that are genetically analogous to 10 different inher
ited cancer syndromes in humans. We describe the variable usefulness o
f the mutant mice as models for human disease. (C) 1998 Elsevier Scien
ce B.V. All rights reserved.