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CEBPA  -  CCAAT/enhancer binding protein (C/EBP), alpha

Homo sapiens

 
 
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Disease relevance of CEBPA

  • Favorable prognostic significance of CEBPA mutations in patients with de novo acute myeloid leukemia: a study from the Acute Leukemia French Association (ALFA) [1].
  • These results identify CEBPA as a key target of the leukemic fusion protein AME and suggest that modulation of CEBPA by CRT may represent a mechanism involved in the differentiation block in AME leukemias [2].
  • Recently, several works have reported the presence of CEBPA-acquired mutations in hematological malignancies [3].
  • Overexpression of CEBPA resulting from the translocation t(14;19)(q32;q13) of human precursor B acute lymphoblastic leukemia [4].
  • Mutations of the myeloid transcription factor CEBPA are not associated with the blast crisis of chronic myeloid leukaemia [5].
  • In concordance with these results, CDDO induces a CEBPA ratio change and differentiation of primary blasts from patients with acute myeloid leukemia (AML) [6].
 

High impact information on CEBPA

  • Dominant-negative mutations of CEBPA, encoding CCAAT/enhancer binding protein-alpha (C/EBPalpha), in acute myeloid leukemia [7].
  • Conditional expression of C/EBPalpha triggers neutrophilic differentiation, and no mature granulocytes are observed in Cebpa-mutant mice [7].
  • In addition, we detected no differences in CEBPA mRNA levels in leukemic blasts of patients carrying the AME translocation (n = 8) compared to acute myeloid leukemia patients with a normal karyotype (n = 9) [2].
  • A recurrent in-frame insertion in a CEBPA transactivation domain is a polymorphism rather than a mutation that does not affect gene expression profiling-based clustering of AML [8].
  • The CEBPA protein is known to regulate the balance between cell proliferation and differentiation during early hematopoietic development and myeloid differentiation [4].
 

Biological context of CEBPA

  • CEBPA mutations were present only in patients belonging to the intermediate cytogenetic risk subgroup and associated with the FAB-M1 subtype (P =.02) [1].
  • PURPOSE: To assess the prognostic relevance of mutations in the CEBPA gene encoding CCAAT/enhancer binding protein alpha (C/EBP alpha) in a large prospective series of younger adults with acute myeloid leukemia (AML) and normal cytogenetics [9].
  • The leukemic fusion gene AML1-MDS1-EVI1 suppresses CEBPA in acute myeloid leukemia by activation of Calreticulin [2].
  • Systematic analysis of CEBPA mutations, in addition to that of alterations in master genes of hematopoiesis, may be useful to assess the prognosis of AML particularly in patients belonging to the 'intermediate' prognostic subgroup [3].
  • The CCAAT/enhancer-binding protein-alpha (CEBPA) is a transcription factor strongly implicated in myelopoiesis through control of proliferation and differentiation of myeloid progenitors [3].
 

Anatomical context of CEBPA

  • Here, we found that conditional expression of core-binding factor beta (CBFB)-SMMHC in U937 cells suppresses CEBPA protein expression and binding activity [10].
  • The transcription factor C/EBPalpha (for CCAAT/enhancer binding protein-alpha; encoded by the gene CEBPA) is crucial for the differentiation of granulocytes [7].
  • Furthermore, we observed that calreticulin (CRT), a putative inhibitor of CEBPA translation, was strongly activated after induction of AME in the cell-line system (14.8-fold) and in AME patient samples (12.2-fold) [2].
  • NPM mutations were detected in 47% of patients and were associated with a high white blood cell count, involvement of the monocytic lineage (M4/M5), and a decreased prevalence of CEBPA mutations [11].
  • We report that CEBPA is activated by juxtaposition to the immunoglobulin gene enhancer upon its rearrangement with the immunoglobulin heavy-chain locus in precursor B-cell acute lymphoblastic leukemia harboring t(14;19)(q32;q13) [4].
 

Associations of CEBPA with chemical compounds

 

Physical interactions of CEBPA

 

Regulatory relationships of CEBPA

 

Other interactions of CEBPA

  • Inhibition of calreticulin by siRNA restored CEBPA levels [10].
  • FLT3 internal tandem duplication (ITD) was found in 5 of 15 CEBPA-mutated as compared with 30 of 119 CEBPA-nonmutated cases tested (P =.54) [1].
  • CONCLUSIONS: Assessment of CEBPA mutations, FLT3-ITD, and BAALC expression permits to split normal-karyotype AML into clinically distinct subgroups [20].
  • CEBPA, PAX5 and PU1 were negative for all cell lines tested except in the three positive BCP-cell lines [21].
  • NPM1 mutations associate inversely with the occurrence of CCAAT/enhancer-binding protein-alpha (CEBPA) and NRAS mutations [22].
 

Analytical, diagnostic and therapeutic context of CEBPA

References

  1. Favorable prognostic significance of CEBPA mutations in patients with de novo acute myeloid leukemia: a study from the Acute Leukemia French Association (ALFA). Preudhomme, C., Sagot, C., Boissel, N., Cayuela, J.M., Tigaud, I., de Botton, S., Thomas, X., Raffoux, E., Lamandin, C., Castaigne, S., Fenaux, P., Dombret, H. Blood (2002) [Pubmed]
  2. The leukemic fusion gene AML1-MDS1-EVI1 suppresses CEBPA in acute myeloid leukemia by activation of Calreticulin. Helbling, D., Mueller, B.U., Timchenko, N.A., Hagemeijer, A., Jotterand, M., Meyer-Monard, S., Lister, A., Rowley, J.D., Huegli, B., Fey, M.F., Pabst, T. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  3. CEBPA point mutations in hematological malignancies. Leroy, H., Roumier, C., Huyghe, P., Biggio, V., Fenaux, P., Preudhomme, C. Leukemia (2005) [Pubmed]
  4. Overexpression of CEBPA resulting from the translocation t(14;19)(q32;q13) of human precursor B acute lymphoblastic leukemia. Chapiro, E., Russell, L., Radford-Weiss, I., Bastard, C., Lessard, M., Struski, S., Cave, H., Fert-Ferrer, S., Barin, C., Maarek, O., Della-Valle, V., Strefford, J.C., Berger, R., Harrison, C.J., Bernard, O.A., Nguyen-Khac, F. Blood (2006) [Pubmed]
  5. Mutations of the myeloid transcription factor CEBPA are not associated with the blast crisis of chronic myeloid leukaemia. Pabst, T., Stillner, E., Neuberg, D., Nimer, S., Willman, C.L., List, A.F., Melo, J.V., Tenen, D.G., Mueller, B.U. Br. J. Haematol. (2006) [Pubmed]
  6. CDDO induces granulocytic differentiation of myeloid leukemic blasts through translational up-regulation of p42 CCAAT enhancer binding protein alpha. Koschmieder, S., D'Alò, F., Radomska, H., Schöneich, C., Chang, J.S., Konopleva, M., Kobayashi, S., Levantini, E., Suh, N., Di Ruscio, A., Voso, M.T., Watt, J.C., Santhanam, R., Sargin, B., Kantarjian, H., Andreeff, M., Sporn, M.B., Perrotti, D., Berdel, W.E., Müller-Tidow, C., Serve, H., Tenen, D.G. Blood (2007) [Pubmed]
  7. Dominant-negative mutations of CEBPA, encoding CCAAT/enhancer binding protein-alpha (C/EBPalpha), in acute myeloid leukemia. Pabst, T., Mueller, B.U., Zhang, P., Radomska, H.S., Narravula, S., Schnittger, S., Behre, G., Hiddemann, W., Tenen, D.G. Nat. Genet. (2001) [Pubmed]
  8. A recurrent in-frame insertion in a CEBPA transactivation domain is a polymorphism rather than a mutation that does not affect gene expression profiling-based clustering of AML. Wouters, B.J., Louwers, I., Valk, P.J., L??wenberg, B., Delwel, R. Blood (2007) [Pubmed]
  9. CEBPA mutations in younger adults with acute myeloid leukemia and normal cytogenetics: prognostic relevance and analysis of cooperating mutations. Fröhling, S., Schlenk, R.F., Stolze, I., Bihlmayr, J., Benner, A., Kreitmeier, S., Tobis, K., Döhner, H., Döhner, K. J. Clin. Oncol. (2004) [Pubmed]
  10. CBFB-SMMHC is correlated with increased calreticulin expression and suppresses the granulocytic differentiation factor CEBPA in AML with inv(16). Helbling, D., Mueller, B.U., Timchenko, N.A., Schardt, J., Eyer, M., Betts, D.R., Jotterand, M., Meyer-Monard, S., Fey, M.F., Pabst, T. Blood (2005) [Pubmed]
  11. Prevalence, clinical profile, and prognosis of NPM mutations in AML with normal karyotype. Boissel, N., Renneville, A., Biggio, V., Philippe, N., Thomas, X., Cayuela, J.M., Terre, C., Tigaud, I., Castaigne, S., Raffoux, E., De Botton, S., Fenaux, P., Dombret, H., Preudhomme, C. Blood (2005) [Pubmed]
  12. CCAAT enhancer-binding protein alpha suppresses the rat placental glutathione S-transferase gene in normal liver. Ikeda, H., Omoteyama, K., Yoshida, K., Nishi, S., Sakai, M. J. Biol. Chem. (2006) [Pubmed]
  13. Anandamide induced PPARgamma transcriptional activation and 3T3-L1 preadipocyte differentiation. Bouaboula, M., Hilairet, S., Marchand, J., Fajas, L., Le Fur, G., Casellas, P. Eur. J. Pharmacol. (2005) [Pubmed]
  14. Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I. Bergeron, A., Jorquera, R., Orejuela, D., Tanguay, R.M. J. Biol. Chem. (2006) [Pubmed]
  15. Thapsigargin suppresses phorbol ester-dependent human involucrin promoter activity by suppressing CCAAT-enhancer-binding protein alpha (C/EBPalpha) DNA binding. Balasubramanian, S., Agarwal, C., Efimova, T., Dubyak, G.R., Banks, E., Welter, J., Eckert, R.L. Biochem. J. (2000) [Pubmed]
  16. Glucocorticoid-mediated induction of CYP3A4 is decreased by disruption of a protein: DNA interaction distinct from the pregnane X receptor response element. El-Sankary, W., Bombail, V., Gibson, G.G., Plant, N. Drug Metab. Dispos. (2002) [Pubmed]
  17. Functional characterization of the human resistin promoter with adipocyte determination- and differentiation-dependent factor 1/sterol regulatory element binding protein 1c and CCAAT enhancer binding protein-alpha. Seo, J.B., Noh, M.J., Yoo, E.J., Park, S.Y., Park, J., Lee, I.K., Park, S.D., Kim, J.B. Mol. Endocrinol. (2003) [Pubmed]
  18. The Herpesvirus saimiri replication and transcription activator acts synergistically with CCAAT enhancer binding protein alpha to activate the DNA polymerase promoter. Wakenshaw, L., Walters, M.S., Whitehouse, A. J. Virol. (2005) [Pubmed]
  19. Corticosteroids: potential beta2-agonist and anticholinergic interactions in chronic obstructive pulmonary disease. Johnson, M. Proceedings of the American Thoracic Society. (2005) [Pubmed]
  20. Risk assessment in patients with acute myeloid leukemia and a normal karyotype. Bienz, M., Ludwig, M., Leibundgut, E.O., Mueller, B.U., Ratschiller, D., Solenthaler, M., Fey, M.F., Pabst, T. Clin. Cancer Res. (2005) [Pubmed]
  21. 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]
  22. Mutations in nucleophosmin (NPM1) in acute myeloid leukemia (AML): association with other gene abnormalities and previously established gene expression signatures and their favorable prognostic significance. Verhaak, R.G., Goudswaard, C.S., van Putten, W., Bijl, M.A., Sanders, M.A., Hugens, W., Uitterlinden, A.G., Erpelinck, C.A., Delwel, R., Löwenberg, B., Valk, P.J. Blood (2005) [Pubmed]
  23. Mutations of CEBPA in acute myeloid leukemia FAB types M1 and M2. Snaddon, J., Smith, M.L., Neat, M., Cambal-Parrales, M., Dixon-McIver, A., Arch, R., Amess, J.A., Rohatiner, A.Z., Lister, T.A., Fitzgibbon, J. Genes Chromosomes Cancer (2003) [Pubmed]
  24. A novel fluorescence-based multiplex PCR assay for rapid simultaneous detection of CEBPA mutations and NPM mutations in patients with acute myeloid leukemias. Lin, L.I., Lin, T.C., Chou, W.C., Tang, J.L., Lin, D.T., Tien, H.F. Leukemia (2006) [Pubmed]
  25. Biallelic mutations in the CEBPA gene and low CEBPA expression levels as prognostic markers in intermediate-risk AML. Barjesteh van Waalwijk van Doorn-Khosrovani, S., Erpelinck, C., Meijer, J., van Oosterhoud, S., van Putten, W.L., Valk, P.J., Berna Beverloo, H., Tenen, D.G., Löwenberg, B., Delwel, R. Hematol. J. (2003) [Pubmed]
  26. Downregulation of cytochromes P450 in growth-stimulated rat hepatocytes: role of c-Myc induction and impaired C/EBP binding to DNA. Tinel, M., Berson, A., Elkahwaji, J., Cresteil, T., Beaune, P., Pessayre, D. J. Hepatol. (2003) [Pubmed]
 
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