The paracaspase MALT1 has a central role in the activation of lymphocytes and other immune cells including myeloid cells, mast cells and NK cells. MALT1 activity is required not only for the immune response, but also for the development of natural Treg cells that keep the immune response in check. The paracaspase MALT1 plays an essential role in the activation of immune cells by specific subtypes of immune receptors, which induce a common signaling pathway leading to the activation of the transcription factor NF-κB. NF-κB target genes include cytokines and anti-apoptotic proteins, which together promote the activation, proliferation and survival of the activated immune cells upon receptor triggering, and thereby allow the efficient generation of an immune response. MALT1 is also involved in the activation of non-immune cells, in which an NF-κB response can be induced by specific G protein-coupled receptors (GPCRs) or the epidermal growth factor (EGF) receptor. MALT1 has an essential role in the immune response and the growth of lymphoma cells with constitutive MALT1 activity, MALT1 could be a rational drug target for immunomodulation and lymphoma treatment.


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