Pax5: the guardian of B cell identity and function.
The transcription factor Pax5 is essential for commitment of lymphoid progenitors to the B lymphocyte lineage. Pax5 fulfils a dual role by repressing B lineage 'inappropriate' genes and simultaneously activating B lineage-specific genes. This transcriptional reprogramming restricts the broad signaling capacity of uncommitted progenitors to the B cell pathway, regulates cell adhesion and migration, induces V(H)-DJ(H) recombination, facilitates (pre-)B cell receptor signaling and promotes development to the mature B cell stage. Conditional Pax5 inactivation in early and late B lymphocytes revealed an essential role for Pax5 in controlling the identity and function of B cells throughout B lymphopoiesis. PAX5 has also been implicated in human B cell malignancies, as it is deregulated by chromosomal translocations in a subset of acute lymphoblastic leukemias and non-Hodgkin lymphomas.
Cobaleda
César
C
Research Institute of Molecular Pathology, Vienna Biocenter, Dr. Bohr-Gasse 7, A-1030 Vienna, Austria.
Schebesta
Alexandra
A
Delogu
Alessio
A
Busslinger
Meinrad
M
D000818:Animals; D001402:B-Lymphocytes / Q000378:metabolism* / Q000473:pathology; D002454:Cell Differentiation / Q000235:genetics*; D019070:Cell Lineage; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D018507:Gene Expression Regulation, Developmental / Q000502:physiology*; D006801:Humans; D051757:PAX5 Transcription Factor / Q000235:genetics / Q000502:physiology*
D051757:PAX5 Transcription Factor; C497281:PAX5 protein, human; C497282:Pax5 protein, mouse