Characterization of a panel of somatic cell hybrids for subregional mapping along 11p and within band 11p13: subdivision of the WAGR complex region

• Published in: Human genetics Vol. 82; no. 2; pp. 171 - 178
• Main Authors: COUILLIN, P, AZOULAY, M, VAN HEYNINGEN, V, JUNIEN, C, HENRY, I, RAVISE, N, GRISARD, M. C, JEANPIERRE, C, BARICHARD, F, METEZEAU, P, CANDELIER, J. J, LEWIS, W
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01-05-1989; Berlin; New York, NY
• Subjects: Antibodies, Monoclonal; Biological and medical sciences; Blotting, Southern; Chromosome Deletion; Chromosome Mapping; Chromosomes, Human, Pair 11 - ultrastructure; Complex syndromes; DNA Probes; Female; Genetic Markers; Humans; Hybrid Cells; Male; Medical genetics; Medical sciences; Syndrome; Chromosomal aberration; Somatic cell; WAGR syndrom; Mental retardation; Genital diseases; Blotting assay; ENT disease; Molecular probe; Abnormal C11 chromosome; Southern blotting; Complex syndrome; Genetic mapping; Human; Urinary system disease; Hybrid cell; Beckwith Wiedemann syndrome; Rodentia; Genital system; Vertebrata; Eye disease; Mammalia; Urinary system; Mouse; Malformation; Abnormal chromosome
• ISSN: 0340-6717; 1432-1203
• DOI: 10.1007/BF00284053

The short arm of chromosome 11 carries genes involved in malformation syndromes, including the aniridia/genitourinary abnormalities/mental retardation (WAGR) syndrome and the Beckwith-Wiedemann syndrome, both of which are associated with an increased risk of childhood malignancy. Evidence comes from constitutional chromosomal aberrations and from losses of heterozygosity, limited to tumor cells, involving regions 11p13 and 11p15. In order to map the genes involved more precisely, we have fused a mouse cell line with cell lines from patients with constitutional deletions or translocations. Characterization of somatic cell hybrids with 11p-specific DNA markers has allowed us to subdivide the short arm into 11 subregions, 7 of which belong to band 11p13. We have thus defined the smallest region of overlap for the Wilms' tumor locus bracketed by the closest proximal and distal breakpoints in two of these hybrids. The region associated with the Beckwith-Wiedemann syndrome spans the region flanked by two 11p15.5 markers, HRAS1 and HBB. These hybrids also represent useful tools for mapping new markers to this region of the human genome.