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Gene Review

PAX5  -  paired box 5

Homo sapiens

Synonyms: ALL3, BSAP
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Disease relevance of PAX5


High impact information on PAX5

  • Pax5 restricts the developmental options of lymphoid progenitors to the B cell lineage by repressing the transcription of lineage-inappropriate genes and simultaneously activating the expression of B-lymphoid signaling molecules [5].
  • Pax-5 (BSAP) recruits Ets proto-oncogene family proteins to form functional ternary complexes on a B-cell-specific promoter [6].
  • Transcription factor Pax5 (BSAP) transactivates the RAG-mediated V(H)-to-DJ(H) rearrangement of immunoglobulin genes [7].
  • In exploring the mechanism for Pax5 regulation of V(H)-to-DJ(H) recombination, we have identified multiple Pax5 binding sites in the coding regions of human and mouse V(H) gene segments [7].
  • The B cell-specific transcription factor program was disrupted by overexpression of the helix-loop-helix proteins ABF-1 and Id2 [8].

Chemical compound and disease context of PAX5

  • In the desmoplastic variant of medulloblastoma, PAX5 expression was restricted to the reticulin-producing proliferating tumor areas containing undifferentiated cells; PAX5 was not expressed in the reticulin-free nonproliferating islands undergoing neuronal differentiation [1].

Biological context of PAX5


Anatomical context of PAX5

  • Blasts from 150 B-cell ALLs showed strong PAX5 nuclear expression, paralleling that of CD79a in the cytoplasm [2].
  • Conversely, PAX5 was not detected in 50 T-cell ALLs, including 20 cases aberrantly coexpressing CD79a [2].
  • Silencing of PAX5 gene by upregulation of B-lymphocyte-induced maturation protein-1 (PRDM1) is essential for terminal differentiation of B cells to plasma cells [4].
  • Using paraffin-embedded bone marrow biopsy sections, we found PAX5/BSAP was expressed in 72% (18/25) of cases overall with an intensity ranging from weak (10, 56%) to strong (8, 44%) [4].
  • PAX5-gene expression is restricted to the mesencephalon-rhombencephalon boundary and the spinal cord [10].

Associations of PAX5 with chemical compounds

  • Cultivation of Pax5(-/-) pro-B cells on OP9 cells expressing the Notch ligand Delta-Like1 (OP9-DL1) in the presence of IL-7 efficiently induced T and NK cell potential [11].
  • Thus, BSAP/Pax5A was constitutively expressed in the multipotent EML cell line, which can be directed toward the myeloid lineage by culture with interleukin-3 (IL-3) and retinoic acid [12].
  • To probe the latter issue, we have created monomeric forms of CREB by fusing CREB to the DNA-binding domain of a protein (B-cell specific activator protein [BSAP]) that binds to DNA as a monomer [13].
  • Together with fracture and densitometry data from the original study, relationships were examined between BMD and serum IGF-I, IGFBP-3, testosterone, estradiol, BSAP, and urine NTx, both at baseline and during treatment with alendronate, to gain possible insights into the pathogenesis of male osteoporosis [14].
  • Whereas BSAP did not change significantly, S-NTX decreased significantly by risedronate treatment [15].

Physical interactions of PAX5

  • We hypothesize that premature Blimp-1 expression coupled to altered and deficient Pax5 expression causes some proliferating B cells to prematurely differentiate to plasma cells in MM [16].
  • The NLS sequence in the central domain of BSAP binds to the C-terminal 98-amino acid fragment of importin alpha1 [17].

Regulatory relationships of PAX5

  • Interestingly, PAX5(+) t(8;21)-AML also expressed CD79a and/or CD19 (major transcriptional targets of PAX5 in B-cells) in 10 of 12 evaluable cases [2].
  • Transient Notch signaling induces NK cell potential in Pax5-deficient pro-B cells [11].

Other interactions of PAX5


Analytical, diagnostic and therapeutic context of PAX5

  • PAX5 transcripts were readily detectable in clinical materials with a wide variety of B-cell neoplasms by reverse transcriptase-mediated polymerase chain reaction (PCR) [21].
  • Electrophoretic mobility shift assays demonstrate that the isoforms possess specific DNA binding activity and recognize the PAX-5 consensus binding sites [22].
  • Although the N-terminal paired (DNA-binding) domain of BSAP also conferred nuclear localization when coupled to green fluorescent protein, this domain did not bind to importin alpha1 in the yeast two-hybrid assay [17].
  • Molecular cloning of t(9;14)(p13;q32) revealed juxtaposition of the PAX5 to the immunoglobulin heavy chain gene (IGH), although breakpoints on both genes were variable [23].
  • RESULTS: PAX5 expression was found in 50 patients with bladder TCC but in no patient from the control group [24].


  1. Deregulated expression of PAX5 in medulloblastoma. Kozmik, Z., Sure, U., Rüedi, D., Busslinger, M., Aguzzi, A. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  2. PAX5 expression in acute leukemias: higher B-lineage specificity than CD79a and selective association with t(8;21)-acute myelogenous leukemia. Tiacci, E., Pileri, S., Orleth, A., Pacini, R., Tabarrini, A., Frenguelli, F., Liso, A., Diverio, D., Lo-Coco, F., Falini, B. Cancer Res. (2004) [Pubmed]
  3. Chromosomal translocations involving paired box transcription factors in human cancer. Barr, F.G. Int. J. Biochem. Cell Biol. (1997) [Pubmed]
  4. Expression of PAX5 in CD20-positive multiple myeloma assessed by immunohistochemistry and oligonucleotide microarray. Lin, P., Mahdavy, M., Zhan, F., Zhang, H.Z., Katz, R.L., Shaughnessy, J.D. Mod. Pathol. (2004) [Pubmed]
  5. Transcriptional control of early B cell development. Busslinger, M. Annu. Rev. Immunol. (2004) [Pubmed]
  6. Pax-5 (BSAP) recruits Ets proto-oncogene family proteins to form functional ternary complexes on a B-cell-specific promoter. Fitzsimmons, D., Hodsdon, W., Wheat, W., Maira, S.M., Wasylyk, B., Hagman, J. Genes Dev. (1996) [Pubmed]
  7. Transcription factor Pax5 (BSAP) transactivates the RAG-mediated V(H)-to-DJ(H) rearrangement of immunoglobulin genes. Zhang, Z., Espinoza, C.R., Yu, Z., Stephan, R., He, T., Williams, G.S., Burrows, P.D., Hagman, J., Feeney, A.J., Cooper, M.D. Nat. Immunol. (2006) [Pubmed]
  8. Intrinsic inhibition of transcription factor E2A by HLH proteins ABF-1 and Id2 mediates reprogramming of neoplastic B cells in Hodgkin lymphoma. Mathas, S., Janz, M., Hummel, F., Hummel, M., Wollert-Wulf, B., Lusatis, S., Anagnostopoulos, I., Lietz, A., Sigvardsson, M., Jundt, F., Jöhrens, K., Bommert, K., Stein, H., Dörken, B. Nat. Immunol. (2006) [Pubmed]
  9. Aberrant promoter methylation of the transcription factor genes PAX5 alpha and beta in human cancers. Palmisano, W.A., Crume, K.P., Grimes, M.J., Winters, S.A., Toyota, M., Esteller, M., Joste, N., Baylin, S.B., Belinsky, S.A. Cancer Res. (2003) [Pubmed]
  10. PAX-genes expression during human embryonic development, a preliminary report. Gérard, M., Abitbol, M., Delezoide, A.L., Dufier, J.L., Mallet, J., Vekemans, M. C. R. Acad. Sci. III, Sci. Vie (1995) [Pubmed]
  11. Transient Notch signaling induces NK cell potential in Pax5-deficient pro-B cells. Carotta, S., Brady, J., Wu, L., Nutt, S.L. Eur. J. Immunol. (2006) [Pubmed]
  12. BSAP/Pax5A expression blocks survival and expansion of early myeloid cells implicating its involvement in maintaining commitment to the B-lymphocyte lineage. Chiang, M.Y., Monroe, J.G. Blood (1999) [Pubmed]
  13. A monomeric derivative of the cellular transcription factor CREB functions as a constitutive activator. Krajewski, W., Lee, K.A. Mol. Cell. Biol. (1994) [Pubmed]
  14. An investigation of the predictors of bone mineral density and response to therapy with alendronate in osteoporotic men. Drake, W.M., Kendler, D.L., Rosen, C.J., Orwoll, E.S. J. Clin. Endocrinol. Metab. (2003) [Pubmed]
  15. Serum NTX is a practical marker for assessing antiresorptive therapy for glucocorticoid treated patients with chronic kidney disease. Hamano, T., Fujii, N., Nagasawa, Y., Isaka, Y., Moriyama, T., Okada, N., Imai, E., Horio, M., Ito, T. Bone (2006) [Pubmed]
  16. Altered mRNA expression of Pax5 and Blimp-1 in B cells in multiple myeloma. Borson, N.D., Lacy, M.Q., Wettstein, P.J. Blood (2002) [Pubmed]
  17. BSAP (Pax5)-importin alpha 1 (Rch1) interaction identifies a nuclear localization sequence. Kovac, C.R., Emelyanov, A., Singh, M., Ashouian, N., Birshtein, B.K. J. Biol. Chem. (2000) [Pubmed]
  18. Distinct promoters mediate the regulation of ebf1 gene expression by interleukin-7 and pax5. Roessler, S., Gy??ry, I., Imhof, S., Spivakov, M., Williams, R.R., Busslinger, M., Fisher, A.G., Grosschedl, R. Mol. Cell. Biol. (2007) [Pubmed]
  19. Transcription factor expression in cell lines derived from natural killer-cell and natural killer-like T-cell leukemia-lymphoma. Matsuo, Y., Drexler, H.G., Harashima, A., Okochi, A., Shimizu, N., Orita, K. Hum. Cell (2004) [Pubmed]
  20. Aberrant expression of ID2, a suppressor of B-cell-specific gene expression, in Hodgkin's lymphoma. Renné, C., Martin-Subero, J.I., Eickernjäger, M., Hansmann, M.L., Küppers, R., Siebert, R., Bräuninger, A. Am. J. Pathol. (2006) [Pubmed]
  21. Expression of the PAX5/BSAP transcription factor in haematological tumour cells and further molecular characterization of the t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma. Hamada, T., Yonetani, N., Ueda, C., Maesako, Y., Akasaka, H., Akasaka, T., Ohno, H., Kawakami, K., Amakawa, R., Okuma, M. Br. J. Haematol. (1998) [Pubmed]
  22. Human Pax-5 C-terminal isoforms possess distinct transactivation properties and are differentially modulated in normal and malignant B cells. Robichaud, G.A., Nardini, M., Laflamme, M., Cuperlovic-Culf, M., Ouellette, R.J. J. Biol. Chem. (2004) [Pubmed]
  23. The t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma. Ohno, H., Ueda, C., Akasaka, T. Leuk. Lymphoma (2000) [Pubmed]
  24. The expression of PAX5, p53 immunohistochemistry and p53 mutation analysis in superficial bladder carcinoma tissue. Correlation with pathological findings and clinical outcome. Babjuk, M., Soukup, V., Mares, J., Dusková, J., Sedlácek, Z., Trková, M., Pecen, L., Dvorácek, J., Hanus, T., Kocvara, R., Novák, J., Povýsil, C. International urology and nephrology. (2002) [Pubmed]
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