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Acute Leukemia

Nebral K, Denk D, Attarbaschi A et al.

St Anna Kinderkrebsforschung, Vienna, Austria.

 Leukemia 2009;23:134–43.

Editor’s note: The gene paired box 5 (PAX5) encodes the transcription factor also known as B cell lineage specific activator protein, which regulates B cell development. Within the hematopoietic system, PAX5 is exclusively expressed in B lymphoid lineage cells and cells must express this marker in order to progress beyond the pro-B cell stage. If pro-B cells lose PAX5 expression, B cell development is arrested at the pro-B cell stage and these committed B cell precursors revert back to progenitor cells with self renewal capacity. PAX5 has recently been shown to be involved in several leukemia-associated chromosomal rearrangements that result in fusion genes encoding chimeric proteins that antagonize PAX5 transcriptional activity.

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