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Transgenic
mice carrying a mutant p53 gene
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develop many
types of cancer, with a high
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proportion of
sarcomas (Lavigueur et al.
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1989). This
observation led various authors
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to study
patients with Li-Fraumeni
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syndrome. This
syndrome presents as a
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familial
association of a broad spectrum of
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cancers
including osteosarcomas, breast
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cancer, soft
tissue sarcoma and leukemias,
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appearing at a
very early age. Statistical
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analysis
predicts that 50 % of these
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individuals
will have a tumor before the age
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of 30, and 90
% before the age of 70. Germ-
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line mutations
in the p53 gene have been
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found in
several families with this syndrome
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(Malkin et
al. 1990; Srivastava et al. 1990). In
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all cases
there is a strict correlation between
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transmission
of the mutant allele and
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development of
a cancer.
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