| Identity |
| Other names | C2TA |
| HGNC | CIITA |
| Location | 16p13 |
| Location_base_pair | Starts at 10878556 and ends at 10926341 bp from pter ( according to hg18-Mar_2006). |
| DNA/RNA |
| Transcription | 6.7 kb mRNA; coding sequence: 3392 bp |
| Protein |
| Description | 1130 amino acids |
| Function | non-DNA-binding transcriptional coactivator; highly regulated; IFN-gamma-inducible; transactivator of the major histocompatibility complex (MHC) class II genes, herein the name; DM and Ii (molecules associated to MHC class II) are also regulated by MHC2TA; MHC2TA also transactivates MHC class I; lymphoid cells of MHC2TA-null mice do not express MHC class II |
| Implicated in |
| Entity | t(3;16)(q27;p13)/ BCL6 |
| Disease | non-Hodgkin lymphoma |
| Prognosis | yet unknown (only 1case available with certainty) |
| Hybrid/Mutated Gene | 5' MHC2TA - 3' BCL6; MHC2TA fuses with the second exon of BCL6 |
| Abnormal Protein | no fusion protein, but promoter exchange |
| Oncogenesis | BCL6 is a transcription repressor; it is supposed that substitution of the promoter of BCL6 may be responsible for BCL6 deregulation |
| Entity | bare lymphocyte syndrome (BLS) |
| Note | at least five complementation groups |
| Disease | severe immunodeficiency due to failure to express MHC class II genes |
| External links |
| Bibliography |
| Complementation cloning of an MHC class II transactivator mutated in hereditary MHC class II deficiency (or bare lymphocyte syndrome). |
| Steimle V, Otten LA, Zufferey M, Mach B |
| Cell. 1993 ; 75 (1) : 135-146. |
| PMID 8402893 |
| CIITA stimulation of transcription factor binding to major histocompatibility complex class II and associated promoters in vivo. |
| Wright KL, Chin KC, Linhoff M, Skinner C, Brown JA, Boss JM, Stark GR, Ting JP |
| Proceedings of the National Academy of Sciences of the United States of America. 1998 ; 95 (11) : 6267-6272. |
| PMID 9600954 |
| The class II transactivator CIITA is a transcriptional integrator. |
| Fontes JD, Kanazawa S, Nekrep N, Peterlin BM |
| Microbes and infection / Institut Pasteur. 1999 ; 1 (11) : 863-869. |
| PMID 10614003 |
| Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples. |
| Yoshida S, Kaneita Y, Aoki Y, Seto M, Mori S, Moriyama M |
| Oncogene. 1999 ; 18 (56) : 7994-7999. |
| PMID 10637510 |
| CIITA is a transcriptional coactivator that is recruited to MHC class II promoters by multiple synergistic interactions with an enhanceosome complex. |
| Masternak K, Muhlethaler-Mottet A, Villard J, Zufferey M, Steimle V, Reith W |
| Genes & development. 2000 ; 14 (9) : 1156-1166. |
| PMID 10809673 |
| Synergistic induction of HLA class I expression by RelA and CIITA. |
| Girdlestone J |
| Blood. 2000 ; 95 (12) : 3804-3808. |
| PMID 10845913 |
| REVIEW articles | automatic search in PubMed |
| Last year publications | automatic search in PubMed |
| Contributor(s) |
| Written | 07-2000 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . MHC2TA (MHC class II transactivator). Atlas Genet Cytogenet Oncol Haematol. July 2000 . URL : http://AtlasGeneticsOncology.org/Genes/MHC2TAID260.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Sun Nov 9 19:43:26 2008 |
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