Bulletin du cancer
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The unique genetic demography of the French Canadian population of Quebec, Canada, has provided a means to study the contribution of BRCA1 and BRCA2, the breast-ovarian cancer susceptibility genes. Here we review BRCA1 and BRCA2 in the context of French Canadian cancer families, a well-characterized founder population known for its contributions to medical genetics. Pathogenic BRCA1 and BRCA2 mutations contribute to a significant proportion of hereditary breast and/or ovarian cancer families of French Canadian descent. ⋯ This effect has been attributed to common founders in the French Canadian population of Quebec who emigrated from France in between 1608 and 1759. It is possible that novel highly penetrant cancer susceptibility genes account for a fraction of the 60 % of BRCA mutation-negative French Canadian cancer families. The continued genetic analysis and phenotypic characterization of French Canadian cancer families is warranted given the large family structure of these families are amenable for classical genome-wide linkage analysis for novel breast and ovarian cancer susceptibility genes.