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Inflammation in bronchiectasis is mediated primarily by activated neutrophils that release NSPs (60)
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Importantly, IGF-1 levels and IGF-1R downstream signaling are suppressed in many chronic disease conditions and likely result in muscle atrophy via the combined effects of altered protein synthesis, UPS activity, autophagy, and muscle regeneration
In contrast, both TDP-43 and FUS are RNA and DNA binding proteins found predominantly in the nucleus, where they regulate transcription, the DNA response, mRNA splicing, RNA stability, micro-RNA biogenesis, translation and transport, and stress granule formation (Cohen et al., 2019