Balanced translocation in a neuroblastoma patient disrupts a cluster of small nuclear RNA UI and tRNA genes in chromosomal band Ip36
✍ Scribed by Pauline van der Drift; Alvin Chan; Geneviève Laureys; Nadine van Roy; Grace Sickmann; Johan den Dunnen; Andries Westerveld; Frank Speleman; Rogier Versteeg
- Book ID
- 102843386
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 630 KB
- Volume
- 14
- Category
- Article
- ISSN
- 1045-2257
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✦ Synopsis
Chromosomal band lp36 probably harbours several neuroblastoma suppressor genes. A neuroblastoma patient has been described with a constitutional balanced translocation, t( I ; I7)(p36;q 12-2 I ). Cytogenetically, no loss of chromosomal material was visible. The I p36 translocation breakpoint could therefore have inactivated one allele of a tumour suppressor gene, thus predisposing the patient to develop neuroblastoma. We localized this breakpoint by pulsed field gel electrophoresis, analysis of yeast artificial chromosomes, and fluorescence in situ hybridization. Here we report that the breakpoint is within a large cluster of small nuclear RNA U I (RNU I) and some tRNA genes (TRE, TRN) on chromosomal band I p36. The size of this cluster is over two megabases and it contains many other locally repeated sequences. Polyadenylated transcripts were identified for some of these sequences. In addition, the cluster is the target for integration of an adenovirus 5/SV40 hybrid virus. The translocation breakpoint maps distal of this viral integration site and proximal of marker PND. Genes Chrornosorn Cancer 14:35-42 (1995).