This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrowReprints and Permissions
Right arrow Copyright Information
Right arrow Books from ASM Press
Right arrow MicrobeWorld
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by DeSandro, A. M.
Right arrow Articles by Boss, J. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by DeSandro, A. M.
Right arrow Articles by Boss, J. M.

 Previous Article  |  Next Article 

Molecular and Cellular Biology, September 2000, p. 6587-6599, Vol. 20, No. 17
0270-7306/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.

Associations and Interactions between Bare Lymphocyte Syndrome Factors

Angela M. DeSandro, Uma M. Nagarajan, and Jeremy M. Boss*

Department of Microbiology and Immunology, Emory University School of Medicine, Atlanta, Georgia 30322

Received 1 December 1999/Returned for modification 12 January 2000/Accepted 26 May 2000

The bare lymphocyte syndrome, a severe combined immunodeficiency due to loss of major histocompatibility complex (MHC) class II gene expression, is caused by inherited mutations in the genes encoding the heterotrimeric transcription factor RFX (RFX-B, RFX5, and RFXAP) and the class II transactivator CIITA. Mutagenesis of the RFX genes was performed, and the properties of the proteins were analyzed with regard to transactivation, DNA binding, and protein-protein interactions. The results identified specific domains within each of the three RFX subunits that were necessary for RFX complex formation, including the ankyrin repeats of RFX-B. DNA binding was dependent on RFX complex formation, and transactivation was dependent on a region of RFX5. RFX5 was found to interact with CIITA, and this interaction was dependent on a proline-rich domain within RFX5. Thus, these studies have defined the protein domains required for the functional regulation of MHC class II genes.

* Corresponding author. Mailing address: Department of Microbiology and Immunology, Emory University School of Medicine, Atlanta, GA 30322. Phone: (404) 727-5973. Fax: (404) 727-3659. E-mail: boss{at}microbio.emory.edu.

Molecular and Cellular Biology, September 2000, p. 6587-6599, Vol. 20, No. 17
0270-7306/00/$04.00+0
Copyright © 2000, American Society for Microbiology. All rights reserved.


This article has been cited by other articles:

  • Seguin-Estevez, Q., De Palma, R., Krawczyk, M., Leimgruber, E., Villard, J., Picard, C., Tagliamacco, A., Abbate, G., Gorski, J., Nocera, A., Reith, W. (2009). The Transcription Factor RFX Protects MHC Class II Genes against Epigenetic Silencing by DNA Methylation. J. Immunol. 183: 2545-2553 [Abstract] [Full Text]  
  • Majumder, P., Gomez, J. A., Chadwick, B. P., Boss, J. M. (2008). The insulator factor CTCF controls MHC class II gene expression and is required for the formation of long-distance chromatin interactions. JEM 205: 785-798 [Abstract] [Full Text]  
  • Voong, L. N., Slater, A. R., Kratovac, S., Cressman, D. E. (2008). Mitogen-activated Protein Kinase ERK1/2 Regulates the Class II Transactivator. J. Biol. Chem. 283: 9031-9039 [Abstract] [Full Text]  
  • Tosi, G., Pilotti, E., Mortara, L., Barbaro, A. D. L., Casoli, C., Accolla, R. S. (2006). Inhibition of human T cell leukemia virus type 2 replication by the suppressive action of class II transactivator and nuclear factor Y. Proc. Natl. Acad. Sci. USA 103: 12861-12866 [Abstract] [Full Text]  
  • Buttice, G., Miller, J., Wang, L., Smith, B. D. (2006). Interferon-{gamma} Induces Major Histocompatibility Class II Transactivator (CIITA), Which Mediates Collagen Repression and Major Histocompatibility Class II Activation by Human Aortic Smooth Muscle Cells. Circ. Res. 98: 472-479 [Abstract] [Full Text]  
  • Niesen, M. I., Osborne, A. R., Yang, H., Rastogi, S., Chellappan, S., Cheng, J. Q., Boss, J. M., Blanck, G. (2005). Activation of a Methylated Promoter Mediated by a Sequence-specific DNA-binding Protein, RFX. J. Biol. Chem. 280: 38914-38922 [Abstract] [Full Text]  
  • Krawczyk, M., Masternak, K., Zufferey, M., Barras, E., Reith, W. (2005). New Functions of the Major Histocompatibility Complex Class II-Specific Transcription Factor RFXANK Revealed by a High-Resolution Mutagenesis Study. Mol. Cell. Biol. 25: 8607-8618 [Abstract] [Full Text]  
  • Wang, A. H., Gregoire, S., Zika, E., Xiao, L., Li, C. S., Li, H., Wright, K. L., Ting, J. P., Yang, X.-J. (2005). Identification of the Ankyrin Repeat Proteins ANKRA and RFXANK as Novel Partners of Class IIa Histone Deacetylases. J. Biol. Chem. 280: 29117-29127 [Abstract] [Full Text]  
  • Sengupta, P., Xu, Y., Wang, L., Widom, R., Smith, B. D. (2005). Collagen {alpha}1(I) Gene (COL1A1) Is Repressed by RFX Family. J. Biol. Chem. 280: 21004-21014 [Abstract] [Full Text]  
  • Muhlethaler-Mottet, A., Krawczyk, M., Masternak, K., Spilianakis, C., Kretsovali, A., Papamatheakis, J., Reith, W. (2004). The S Box of Major Histocompatibility Complex Class II Promoters Is a Key Determinant for Recruitment of the Transcriptional Co-activator CIITA. J. Biol. Chem. 279: 40529-40535 [Abstract] [Full Text]  
  • Osborne, A. R., Zhang, H., Fejer, G., Palubin, K. M., Niesen, M. I., Blanck, G. (2004). Oct-1 Maintains an Intermediate, Stable State of HLA-DRA Promoter Repression in Rb-defective Cells: AN Oct-1-CONTAINING REPRESSOSOME THAT PREVENTS NF-Y BINDING TO THE HLA-DRA PROMOTER. J. Biol. Chem. 279: 28911-28919 [Abstract] [Full Text]  
  • Nagarajan, U. M., Long, A. B., Harreman, M. T., Corbett, A. H., Boss, J. M. (2004). A Hierarchy of Nuclear Localization Signals Governs the Import of the Regulatory Factor X Complex Subunits and MHC Class II Expression. J. Immunol. 173: 410-419 [Abstract] [Full Text]  
  • Xu, Y., Wang, L., Buttice, G., Sengupta, P. K., Smith, B. D. (2003). Interferon {gamma} Repression of Collagen (COL1A2) Transcription Is Mediated by the RFX5 Complex. J. Biol. Chem. 278: 49134-49144 [Abstract] [Full Text]  
  • Sisk, T. J., Nickerson, K., Kwok, R. P. S., Chang, C.-H. (2003). Phosphorylation of class II transactivator regulates its interaction ability and transactivation function. Int Immunol 15: 1195-1205 [Abstract] [Full Text]  
  • Pai, R. K., Convery, M., Hamilton, T. A., Boom, W. H., Harding, C. V. (2003). Inhibition of IFN-{gamma}-Induced Class II Transactivator Expression by a 19-kDa Lipoprotein from Mycobacterium tuberculosis: A Potential Mechanism for Immune Evasion. J. Immunol. 171: 175-184 [Abstract] [Full Text]  
  • Kanaseki, T., Ikeda, H., Takamura, Y., Toyota, M., Hirohashi, Y., Tokino, T., Himi, T., Sato, N. (2003). Histone Deacetylation, But Not Hypermethylation, Modifies Class II Transactivator and MHC Class II Gene Expression in Squamous Cell Carcinomas. J. Immunol. 170: 4980-4985 [Abstract] [Full Text]  
  • Das, S., Lin, J.-H., Papamatheakis, J., Sykulev, Y., Tsichlis, P. N. (2002). Differential Splicing Generates Tvl-1/RFXANK Isoforms with Different Functions. J. Biol. Chem. 277: 45172-45180 [Abstract] [Full Text]  
  • Jabrane-Ferrat, N., Nekrep, N., Tosi, G., Esserman, L. J., Peterlin, B. M. (2002). Major Histocompatibility Complex Class II Transcriptional Platform: Assembly of Nuclear Factor Y and Regulatory Factor X (RFX) on DNA Requires RFX5 Dimers. Mol. Cell. Biol. 22: 5616-5625 [Abstract] [Full Text]  
  • Pai, R. K., Askew, D., Boom, W. H., Harding, C. V. (2002). Regulation of Class II MHC Expression in APCs: Roles of Types I, III, and IV Class II Transactivator. J. Immunol. 169: 1326-1333 [Abstract] [Full Text]  
  • Mudhasani, R., Fontes, J. D. (2002). The Class II Transactivator Requires brahma-Related Gene 1 To Activate Transcription of Major Histocompatibility Complex Class II Genes. Mol. Cell. Biol. 22: 5019-5026 [Abstract] [Full Text]  
  • Morris, A. C., Beresford, G. W., Mooney, M. R., Boss, J. M. (2002). Kinetics of a Gamma Interferon Response: Expression and Assembly of CIITA Promoter IV and Inhibition by Methylation. Mol. Cell. Biol. 22: 4781-4791 [Abstract] [Full Text]  
  • Holling, T. M., van der Stoep, N., Quinten, E., van den Elsen, P. J. (2002). Activated Human T Cells Accomplish MHC Class II Expression Through T Cell-Specific Occupation of Class II Transactivator Promoter III. J. Immunol. 168: 763-770 [Abstract] [Full Text]  
  • Gobin, S. J. P., van Zutphen, M., Westerheide, S. D., Boss, J. M., van den Elsen, P. J. (2001). The MHC-Specific Enhanceosome and Its Role in MHC Class I and {beta}2-Microglobulin Gene Transactivation. J. Immunol. 167: 5175-5184 [Abstract] [Full Text]  
  • Peretti, M., Villard, J., Barras, E., Zufferey, M., Reith, W. (2001). Expression of the Three Human Major Histocompatibility Complex Class II Isotypes Exhibits a Differential Dependence on the Transcription Factor RFXAP. Mol. Cell. Biol. 21: 5699-5709 [Abstract] [Full Text]  
  • Nekrep, N., Geyer, M., Jabrane-Ferrat, N., Peterlin, B. M. (2001). Analysis of Ankyrin Repeats Reveals How a Single Point Mutation in RFXANK Results in Bare Lymphocyte Syndrome. Mol. Cell. Biol. 21: 5566-5576 [Abstract] [Full Text]  
  • Sisk, T. J., Roys, S., Chang, C.-H. (2001). Self-Association of CIITA and Its Transactivation Potential. Mol. Cell. Biol. 21: 4919-4928 [Abstract] [Full Text]  
  • Li, G., Harton, J. A., Zhu, X., Ting, J. P.-Y. (2001). Downregulation of CIITA Function by Protein Kinase A (PKA)-Mediated Phosphorylation: Mechanism of Prostaglandin E, Cyclic AMP, and PKA Inhibition of Class II Major Histocompatibility Complex Expression in Monocytic Lines. Mol. Cell. Biol. 21: 4626-4635 [Abstract] [Full Text]  
  • Linhoff, M. W., Harton, J. A., Cressman, D. E., Martin, B. K., Ting, J. P.-Y. (2001). Two Distinct Domains within CIITA Mediate Self-Association: Involvement of the GTP-Binding and Leucine-Rich Repeat Domains. Mol. Cell. Biol. 21: 3001-3011 [Abstract] [Full Text]  
  • Lennon-Dumenil, A.-M., Barbouche, M.-R., Vedrenne, J., Prod'Homme, T., Bejaoui, M., Ghariani, S., Charron, D., Fellous, M., Dellagi, K., Alcaide-Loridan, C. (2001). Uncoordinated HLA-D Gene Expression in a RFXANK-Defective Patient with MHC Class II Deficiency. J. Immunol. 166: 5681-5687 [Abstract] [Full Text]  


Copyright © 2009 by the American Society for Microbiology.   For an alternate route to Journals.ASM.org, visit: http://intl-journals.asm.org | More Info»