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Balanced translocation in a neuroblastoma patient disrupts a cluster of small nuclear RNA UI and tRNA genes in chromosomal band Ip36
Author(s) -
van der Drift Pauline,
Chan Alvin,
Laureys Geneviève,
van Roy Nadine,
Sickmann Grace,
den Dunnen Johan,
Westerveld Andries,
Speleman Frank,
Versteeg Rogier
Publication year - 1995
Publication title -
genes, chromosomes and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.754
H-Index - 119
eISSN - 1098-2264
pISSN - 1045-2257
DOI - 10.1002/gcc.2870140107
Subject(s) - chromosomal translocation , breakpoint , biology , gene , microbiology and biotechnology , fluorescence in situ hybridization , neuroblastoma , genetics , chromosomal region , polyadenylation , rna , chromosome , cell culture
Chromosomal band Ip36 probably harbours several neuroblastoma suppressor genes. A neuroblastoma patient has been described with a constitutional balanced translocation, t(1;17)(p36;q12‐21). Cytogenetically, no loss of chromosomal material was visible. The Ip36 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 U1 ( RNU1 ) and some tRNA genes ( TRE, TRN ) on chromosomal band 1p36. 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.