IRF4
| Interferon Regulatory Factor 4 | ||
|---|---|---|
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| other names |
MUM1; LSIRF; SHEP8; NF-EM5 |
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| Properties of human protein | ||
| Mass / length primary structure | 451 amino acids | |
| Identifier | ||
| External IDs | ||
The Interferon Regulatory Factor 4 (IRF4) (dt. Interferon regulatory factor 4 ), also known as MUM1 (of English. Multiple Myeloma Oncogene 1 ) is a transcription factor , usually by way of in lymphocytes is expressed.
Role in B-cell activation
As soon as the B cell recognizes a suitable antigen via the B cell receptor , the transcription of IRF4 is increased via NFκB . This in turn activates MYC and PRDM1 and inhibits BCL6. While feedback to IRF4 and PRDM1 takes place via MYC and BCL6, the latter ensures that the antiproliferative and antitumor effects of interferon are inhibited by inhibiting interferon-β. As a result, the proliferation and thus the degeneration tendency of the B-cell is increased. Physiologically, this effect is used to intensify the fight against the recognized antigen, but if it is activated excessively, it can also lead to the development of tumors.
Large B-cell lymphoma with IRF4 rearrangement
With the revision of the WHO classification of lymphoid neoplasias in 2016, the entity of large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) was introduced, which combines properties of diffuse large-cell B-cell lymphoma and follicular lymphoma . This rare, indolent B-cell non-Hodgkin lymphoma occurs mainly in children and adolescents. It is molecularly characterized by a translocation of IRF4; The gene locus of the heavy immunoglobulin chain (IGH) usually serves as the translocation partner, and less often the light chains (IGK or IGL). Linked to the translocation is a mutation in the IRF4 gene. The prognosis of the LBCL-IRF4 is good, the 10-year survival rate of 80% is significantly higher than that of the classic DLBCL .
Interactions
IRF4 has been shown to interact with the following additional gene products:
- NFATC2
- SPI1
- STAT6
Individual evidence
- ↑ IRF4 interferon regulatory factor 4 (Homo sapiens (human)) Gene, NCBI. Retrieved February 21, 2020.
- ↑ Ramis-Zaldiva JE et al .: IRF4-rearranged Large B-cell lymphoma (LBCL) has a genomic profile distinct to other LBCL in children and young adults In: Blood 2019.
- ↑ Martín-Subero, JI et al .: Interphase FISH assays for the detection of translocations with breakpoints in immunoglobulin light chain loci In: International Journal of Cancer , Volume 98, 2002.