The chromosomal translocation t(X;14)(q28;q11) in T-cell pro-lymphocytic leukaemia breaks within one gene and activates another.
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Fisch P, Forster A, Sherrington PD, Dyer MJ, Rabbitts TH
The chromosomal translocation t(X;14)(q28;q11) in T-cell pro-lymphocytic leukaemia breaks within one gene and activates another.
Oncogene. 1993 Dec;8(12):3271-6.
- PubMed ID
- 8247530 [ View in PubMed]
- Abstract
Chromosomal translocation t(X;14)(q28;q11) has been observed in patients with pro-lymphocytic T-cell leukaemia (T-PLL). In two cases of T-PLL, one of which was associated with Ataxia telangiectasia (AT), the chromosomal break occurred in two different introns of a gene c6.1A, located at the Xq28 locus. Fusion transcripts, consisting of 5' sequences of c6.1A and the TCR alpha constant (C) region, were expressed at high levels in the leukaemic cells from both patients, but in only one case did this fusion generate an in-frame c6.1A-C alpha mRNA. However, the breaks within c6.1A seem to affect another gene, c6.1B, which is transcribed from the same CpG rich island as c6.1A but in the opposite transcriptional orientation. The c6.1B gene is not damaged by the translocation but is transcribed in both T-PLL cases. Furthermore, c6.1B may lack protein coding capacity and thus this translocation might result in a novel mechanism in tumorigenesis. In any event, this is the first cloned gene which is implicated in pathogenesis of chronic/pro-lymphocytic leukaemia of the T-cell lineage.