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A tumor suppressor locus in familial and sporadic chordoma maps to 1p36

✍ Scribed by Monica Miozzo; Leda Dalprà; Paola Riva; Marinella Volontà; Fabio Macciardi; Sergio Pericotti; M. Grazia Tibiletti; Michele Cerati; Klaus Rohde; Lidia Larizza; A. Maria Fuhrman Conti

Publisher: John Wiley and Sons
Year: 2000
Tongue: French
Weight: 150 KB
Volume: 87
Category: Article
ISSN: 0020-7136
DOI: 10.1002/1097-0215(20000701)87:1<68::aid-ijc10>3.0.co;2-v

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✦ Synopsis

Previous cytogenetic/FISH data have demonstrated 1p36 deletions in a relapsing familial clivus chordoma developed by a patient who has 2 daughters, respectively affected with childhood astrocytoma and clivus chordoma. Using an approach that combined the LOH (loss of heterozygosity) study of the father chordoma and the daughter astrocytoma and a segregation analysis from parents to sibs using 17 CA-repeats spanning 1p36.32-1p36.11, we mapped the cancer susceptibility locus in this family to the 1p36 region. The LOH and haplotype information was elaborated using a pairwise linkage analysis that gave a maximum lod score of 1.2. Additional LOH data relating to 6 sporadic chordomas allowed us to define an SRO (the smallest region of overlapping loss) of about 25 cM from D1S2845 (1p36.31) to D1S2728 (1p36.13). Our overall findings converge on mapping to 1p36 a tumorsuppressor gene involved in familial and sporadic chordoma.

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