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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.