Article
An inherited <i>p53</i> mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma
31 Jul 2001
Abstract excerpt
The incidence of pediatric adrenal cortical carcinoma (ACC) in southern Brazil is 10-15 times higher than that of pediatric ACC worldwide. Because childhood ACC is associated with Li-Fraumeni syndrome, we examined the cancer history and p53 status of 36 Brazilian patients and their families. Remarkably, 35 of 36 patients had an identical germ-line point mutation of p53 encoding an R337H amino acid substitution....
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