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Genetic Mapping of a Third Li-Fraumeni Syndrome Predisposition Locus to Human Chromosome 1q23

Sections of ¹ Cancer Genetics and ² Clinical Cancer Genetics, Department of Molecular Genetics and ³ Department of Epidemiology, University of Texas M.D. Anderson Cancer Center, Houston, Texas and ⁴ Human Cancer Genetics Program, Department of Molecular Virology, Immunology and Medical Genetics, Comprehensive Cancer Center, Ohio State University, Columbus, Ohio

Requests for reprints: Ralf Krahe, Section of Cancer Genetics, Unit 11, Department of Molecular Genetics, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4009. Phone: 713-792-2071; Fax: 713-792-8382; E-mail: RalfKrahe{at}mail.mdanderson.org..

Li-Fraumeni syndrome (LFS) is a clinically and genetically heterogeneous inherited cancer syndrome. Most cases (70%) identified and characterized to date are associated with dominantly inherited germ line mutations in the tumor suppressor gene TP53 (p53) in chromosome 17p13.1. In a subset of non-p53 patients with LFS, CHEK2 in chromosome 22q11 has been identified as another predisposing locus. Studying a series of non-p53 LFS kindred, we have shown that there is additional genetic heterogeneity in LFS kindred with inherited predisposition at loci other than p53 or CHEK2. Using a genome-wide scan for linkage with complementing parametric and nonparametric analysis methods, we identified linkage to a region of approximately 4 cM in chromosome 1q23, a genomic region not previously implicated in this disease. Identification ofa third predisposing gene and its underlying mutation(s) should provide insight into other genetic events that predispose to the genesis of the diverse tumor types associated with LFS and its variants.

Key Words: Familial and hereditary cancers • Li-Fraumeni Syndrome (LFS) • Sarcoma/soft-tissue malignancies • Cancer susceptibility genes • Human chromosome 1

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