Paracaspase MALT1 deficiency protects mice from autoimmune-mediated demyelination

C Mc Guire, P Wieghofer, L Elton… - The Journal of …, 2013 - journals.aai.org
C Mc Guire, P Wieghofer, L Elton, D Muylaert, M Prinz, R Beyaert, G van Loo
The Journal of Immunology, 2013journals.aai.org
The paracaspase MALT 1 is a major player in lymphocyte activation and proliferation.
MALT1 mediates Ag-induced signaling to the transcription factor NF-κB by functioning both
as a scaffold protein and cysteine protease. We studied the role of MALT1 in the
development of experimental autoimmune encephalomyelitis (EAE), an animal model of
multiple sclerosis. MALT1-knockout mice did not develop any clinical symptoms of EAE. In
addition, lymphocyte and macrophage infiltration into the spinal cord was absent in MALT1 …
Abstract
The paracaspase MALT 1 is a major player in lymphocyte activation and proliferation. MALT1 mediates Ag-induced signaling to the transcription factor NF-κB by functioning both as a scaffold protein and cysteine protease. We studied the role of MALT1 in the development of experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis. MALT1-knockout mice did not develop any clinical symptoms of EAE. In addition, lymphocyte and macrophage infiltration into the spinal cord was absent in MALT1-knockout mice, as were demyelination and proinflammatory gene expression. Adoptive transfer experiments showed that MALT1 deficiency in splenocytes is sufficient for EAE resistance. Moreover, autoreactive T cell activation was severely impaired in MALT1-deficient T cells, suggesting the inability of MALT1-deficient effector T cells to induce demyelinating inflammation in the CNS. Finally, the MALT1 substrates A20 and CYLD were completely processed in wild-type T cells during EAE, which was partially impaired in MALT1-deficient T cells, suggesting a contribution of MALT1 proteolytic activity in T cell activation and EAE development. Together, our data indicate that MALT1 may be an interesting therapeutic target in the treatment of multiple sclerosis.
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