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COP9 signalosome complex subunit 3 (COPS3) is a component of the COP9 signalosome (CSN), a multiprotein complex involved in the regulation of multiple cellular and developmental processes in eukaryotes. COPS3 has kinase activity and is known to phosphorylate several regulators of signal transduction, including I-kappa-B-alpha, p105, and c-Jun, thus playing a role in cell cycle regulation, ubiquitin-mediated proteolysis, and DNA damage response. It is a PCI (Proteasome, COP9 signalosome, and eIF3) domain-containing protein and localizes predominantly to the cytoplasm and nucleus. Mutations and dysregulation of COPS3 have been implicated in cancer and genetic syndromes, notably Smith-Magenis syndrome due to its genomic location.
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