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However, excessive or aberrant production of inflammatory mediators caused by dysregulated PRR signaling pathways could contribute to the progression of various diseases ( 4.1 Autoinflammatory diseases Autoinflammatory diseases comprise a diverse group of disorders driven by innate immune system dysregulation ( cataloging more than 50 monogenic disorders to date, with additional categories continually emerging ( Table 1 )

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One of the leading mechanisms of astrocyte-associated neurotoxicity is associated with severe decrease in the expression of glutamate transporters, the presence of which is reduced, in ALS patients, to only ~10% of the levels in healthy subjects

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