450-VCP Drive Pathogenic Nuclear Pore Complex Degradation

This research identifies Valosin-containing protein (VCP) as a critical regulator of nuclear pore complex (NPC) integrity in neurons and muscle cells. Under healthy conditions, VCP uses the Ufd1-Npl4 cofactor to remove and degrade specific nucleoporins, maintaining cellular balance. However, pathogenic VCP mutations cause a toxic gain-of-function where the protein excessively strips these essentia...去小宇宙查看完整单集简介
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