Juxtaposition of the T-Cell Receptor α -Chain Locus (14q11) and a Region (14q32) of Potential Importance in Leukemogenesis by a 14;14 Translocation in a Patient with T-Cell Chronic Lymphocytic Leukemia and Ataxia-Telangiectasia
Abstract
We describe a t(14;14)(q11;q32) translocation in a patient with T-cell chronic lymphocytic leukemia and ataxia-telangiectasia (AT). By using a battery of joining (J)-segment probes from the T-cell receptor (TCR) alpha-chain locus TCRA, three distinct J alpha rearrangements were observed. One rearrangement reflected a normal TCRA variable (V) region V alpha-to-J alpha recombination. The second rearrangement was caused by the translocation even itself, which joined a DNA segment from 14q32 centromeric to the immunoglobulin heavy chain locus (IGH) and a J alpha gene located approximately 75 kilobases (kb) 5' of the TCRA constant region gene (C alpha). A third rearrangement involved a 17-kb internal deletion 3' to the translocation, a rearrangement within the J alpha locus that has been observed once before in a patient with AT. Analysis of these three rearrangements underscores the increase in aberrant locus-specific recombination in lymphocytes from patients with AT. Furthermore, these studies support the view that a growth-effecting gene is present in the 14q32 region that participates in the leukemogenic process.
- Publication:
-
Proceedings of the National Academy of Science
- Pub Date:
- December 1988
- DOI:
- 10.1073/pnas.85.23.9287
- Bibcode:
- 1988PNAS...85.9287D