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Title: Pax5  
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Subject: Pax genes, Transcription factors, Haematopoiesis, NeuroD, EMX homeogene
Collection: Transcription Factors
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Paired box 5

PDB rendering based on 1k78.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols  ; ALL3; BSAP
External IDs GeneCards:
RNA expression pattern
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

Paired box protein Pax-5 is a protein that in humans is encoded by the PAX5 gene.[1][2][3] The PAX5 gene is a member of the paired box (PAX) family of transcription factors. The central feature of this gene family is a novel, highly conserved DNA-binding motif, known as the paired box. The PAX proteins are important regulators in early development, and alterations in the expression of their genes are thought to contribute to neoplastic transformation. The PAX5 gene encodes the B-cell lineage specific activator protein (BSAP) that is expressed at early, but not late stages of B-cell differentiation. Its expression has also been detected in developing CNS and testis, therefore, PAX5 gene product may not only play an important role in B-cell differentiation, but also in neural development and spermatogenesis. The PAX5 gene is located in chromosome 9p13 region, which is involved in t(9;14)(p13;q32) translocations recurring in small lymphocytic lymphomas of the plasmacytoid subtype, and in derived large-cell lymphomas. This translocation brings the potent E-mu enhancer of the IgH gene into close proximity of the PAX5 promoters, suggesting that the deregulation of PAX5 gene transcription contributes to the pathogenesis of these lymphomas. A transcript variant arising as a consequence of alternative promoter usage, and containing a different coding exon 1(B), has been described, however, its full-length nature is not known.[3]


  • Pathology 1
  • Interactions 2
  • See also 3
  • References 4
  • Further reading 5
  • External links 6


Up to 97% of the Reed-Sternberg cells of Hodgkins lymphoma express Pax-5.[4]


PAX5 has been shown to interact with TLE4[5][6] and Death associated protein 6.[7]

See also


  1. ^ Adams B, Dorfler P, Aguzzi A, Kozmik Z, Urbanek P, Maurer-Fogy I, Busslinger M (October 1992). "Pax-5 encodes the transcription factor BSAP and is expressed in B lymphocytes, the developing CNS, and adult testis". Genes Dev 6 (9): 1589–607.  
  2. ^ Pilz AJ, Povey S, Gruss P, Abbott CM (March 1993). "Mapping of the human homologs of the murine paired-box-containing genes". Mamm Genome 4 (2): 78–82.  
  3. ^ a b "Entrez Gene: PAX5 paired box gene 5 (B-cell lineage specific activator)". 
  4. ^ Torlakovic, E; Torlakovic G; Nguyen PL; Brunning RD; Delabie J. (October 2002). "The value of anti-pax-5 immunostaining in routinely fixed and paraffin-embedded sections: a novel pan pre-B and B-cell marker". Am J Surg Pathol 26 (10): 1343–50.  
  5. ^ Eberhard, D; Jiménez G; Heavey B; Busslinger M (May 2000). "Transcriptional repression by Pax5 (BSAP) through interaction with corepressors of the Groucho family". EMBO J. (ENGLAND) 19 (10): 2292–303.  
  6. ^ Milili, Michèle; Gauthier Laurent; Veran Julie; Mattei Marie-Geneviève; Schiff Claudine (August 2002). "A new Groucho TLE4 protein may regulate the repressive activity of Pax5 in human B lymphocytes". Immunology (England) 106 (4): 447–55.  
  7. ^ Emelyanov, Alexander V; Kovac Cecilia R; Sepulveda Manuel A; Birshtein Barbara K (March 2002). "The interaction of Pax5 (BSAP) with Daxx can result in transcriptional activation in B cells". J. Biol. Chem. (United States) 277 (13): 11156–64.  

Further reading

  • Hagman J, Wheat W, Fitzsimmons D, et al. (1999). "Pax-5/BSAP: regulator of specific gene expression and differentiation in B lymphocytes.". Curr. Top. Microbiol. Immunol. 245 (1): 169–94.  
  • Calame KL, Lin KI, Tunyaplin C (2003). "Regulatory mechanisms that determine the development and function of plasma cells.". Annu. Rev. Immunol. 21: 205–30.  
  • Carotta S, Holmes ML, Pridans C, Nutt SL (2007). "Pax5 maintains cellular identity by repressing gene expression throughout B cell differentiation.". Cell Cycle 5 (21): 2452–6.  
  • Stapleton P, Weith A, Urbánek P, et al. (1995). "Chromosomal localization of seven PAX genes and cloning of a novel family member, PAX-9.". Nat. Genet. 3 (4): 292–8.  
  • Busslinger M, Klix N, Pfeffer P, et al. (1996). "Deregulation of PAX-5 by translocation of the Emu enhancer of the IgH locus adjacent to two alternative PAX-5 promoters in a diffuse large-cell lymphoma.". Proc. Natl. Acad. Sci. U.S.A. 93 (12): 6129–34.  
  • Iida S, Rao PH, Nallasivam P, et al. (1996). "The t(9;14)(p13;q32) chromosomal translocation associated with lymphoplasmacytoid lymphoma involves the PAX-5 gene.". Blood 88 (11): 4110–7.  
  • Kaneko H, Ariyasu T, Inoue R, et al. (1998). "Expression of Pax5 gene in human haematopoietic cells and tissues: comparison with immunodeficient donors.". Clin. Exp. Immunol. 111 (2): 339–44.  
  • Verkoczy LK, Berinstein NL (1998). "Isolation of genes negatively or positively co-expressed with human recombination activating gene 1 (RAG1) by differential display PCR (DD RT-PCR).". Nucleic Acids Res. 26 (19): 4497–507.  
  • Wheat W, Fitzsimmons D, Lennox H, et al. (1999). "The highly conserved beta-hairpin of the paired DNA-binding domain is required for assembly of Pax-Ets ternary complexes.". Mol. Cell. Biol. 19 (3): 2231–41.  
  • Eberhard D, Busslinger M (1999). "The partial homeodomain of the transcription factor Pax-5 (BSAP) is an interaction motif for the retinoblastoma and TATA-binding proteins.". Cancer Res. 59 (7 Suppl): 1716s–1724s; discussion 1724s–1725s.  
  • Libermann TA, Pan Z, Akbarali Y, et al. (1999). "AML1 (CBFalpha2) cooperates with B cell-specific activating protein (BSAP/PAX5) in activation of the B cell-specific BLK gene promoter". J. Biol. Chem. 274 (35): 24671–6.  
  • Nutt SL, Heavey B, Rolink AG, Busslinger M (1999). "Commitment to the B-lymphoid lineage depends on the transcription factor Pax5". Nature 401 (6753): 556–62.  
  • Rolink AG, Nutt SL, Melchers F, Busslinger M (1999). "Long-term in vivo reconstitution of T-cell development by Pax5-deficient B-cell progenitors". Nature 401 (6753): 603–6.  
  • Kovac CR, Emelyanov A, Singh M, et al. (2000). "BSAP (Pax5)-importin alpha 1 (Rch1) interaction identifies a nuclear localization sequence". J. Biol. Chem. 275 (22): 16752–7.  
  • Eberhard D, Jiménez G, Heavey B, Busslinger M (2000). "Transcriptional repression by Pax5 (BSAP) through interaction with corepressors of the Groucho family". EMBO J. 19 (10): 2292–303.  
  • Roberts EC, Deed RW, Inoue T, et al. (2001). "Id helix-loop-helix proteins antagonize pax transcription factor activity by inhibiting DNA binding". Mol. Cell. Biol. 21 (2): 524–33.  
  • Pasqualucci L, Neumeister P, Goossens T, et al. (2001). "Hypermutation of multiple proto-oncogenes in B-cell diffuse large-cell lymphomas". Nature 412 (6844): 341–6.  

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