B‐cell precursor acute lymphoblastic leukemia (BCP‐ALL) is a common malignancy associated with variable chromosomal translocations, leading to fusion proteins of unknown function. To investigate how such translocations contribute to the development of BCP‐ALL , Smeenk et al (2017) generated mouse models for Pax5 fusion proteins. The results show that a PAX5 fusion is required for BCP‐ALL development by preventing B‐cell differentiation and retaining cells in an arrested progenitor stage. The occurrence of further genetic aberrations eventually results in oncogenic transformation and proliferation of the arrested cells, triggering the onset of leukemia.
See also: L Smeenk et al (March 2017)
B‐cell precursor acute lymphoblastic leukemia (BCP‐ALL) is the most common malignancy in children, and adults are also often diagnosed with this disease. Despite good prognosis in pediatric patient outcomes, at least 15% of patients suffer from relapse (Locatelli et al, 2012). Therefore, a deep understanding of the molecular mechanisms underlying the onset of this disease is needed to improve the current therapeutic approaches.
The commitment into the B‐cell lineage and the subsequent cell fate decisions are regulated through different checkpoints that involve a complex transcriptional regulation. The paired box domain gene 5 (PAX5) is one of the crucial transcription factors required for …
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