Article
[Li-Fraumeni syndrome: update, new data and guidelines for clinical management].
Bulletin du cancer - 1 Jun 2001
Frebourg T, Abel A, Bonaiti-Pellie C, Brugières L, Berthet P, Bressac-de Paillerets B, Chevrier A, Chompret A, Cohen-Haguenauer O, Delattre O, Feingold J, Feunteun J, Frappaz D, Fricker J P, Gesta P, Jonveaux P, Kalifa C, Lasset C, Leheup B, Limacher J M, Longy M, Nogues C, Oppenheim D, Sommelet D, Soubrier F, Stoll C, Stoppa-Lyonnet D, Tristant H
Abstract excerpt
The Li-Fraumeni syndrome (LFS) is an inherited form of cancer, affecting children and young adults, and characterized by a wide spectrum of tumors, including soft-tissue and bone sarcomas, brain tumours, adenocortical tumours and premenopausal breast cancers. In most of the families, LFS results from germline mutations of the tumor suppressor TP53 gene encoding a transcriptional factor able to regulate cell cycle...
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