In common with other factors involved in T-ALL, the normal expression of TLX3 (T-Cell Leukaemia Homeobox 3, previously known as HOX11L2) can be disrupted by a chromosomal translocation1.
Unlike TLX1 (HOX11), however, the dysregulation of TLX3 is not brought about by close juxtaposition with T-cell receptor genes, instead, it is brought into contact with another gene which is highly expressed in normal T-cell differentiation, BCL11B (B-Cell Lymphoma 11B, or CTIP2) on chromosome 14q32.11. The translocation t(5;14)(q35;q32) is generally cryptic and the breakpoint does not actually disrupt TLX3 but, in the majority of cases, occurs within or downstream of the RANBP17 (Ran-Binding Protein 17) gene2. However, this gene is very close to TLX3 and though RANBP17 expression is not affected by the translocation, TLX3 expression is. Dysregulated expression of TLX3 is observed in around 20% of paediatric cases and around 13% of adult T-ALL patients3,4.
1. Bernard OA et al., Leukaemia 2001;15:1495-504
2. Van Zutven et al., Haematologica 2004;89:671-8
3. Van Vlierberghe et al., Leukemia. 2008 Apr;22(4):762-70
4. Borghini et al., Mol Cancer Res. 2006 Sep;4(9):635-43
- Area of Interest*
This product is intended to be used on Carnoy’s solution (3:1 methanol/acetic acid) fixed haematological samples.
*Disease information supported by the literature and is not a reflection of the intended purpose of this product.