Cockayne Syndrome (CS) is characterized by premature aging due to mutations in ERCC6 or ERCC8 genes encoding CSA and CSB proteins. Loss of CSA function causes NE defects, reduced LEMD2-lamin A/C complexes, and increased actin stress fibers. Disruption of the LINC complex rescues NE phenotypes. RNA-seq analysis reveals transcriptional deregulation of cytoskeletal proteins in CS-A cells. Actin polymerization contributes to NE abnormalities, while SUN1 depletion improves NE integrity. Activation of cGAS/STING pathway upon NE ruptures is observed in CS-A cells.
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by Larrieu,D., ... at www.biorxiv.org 12-14-2023
https://www.biorxiv.org/content/10.1101/2023.12.14.571633v1Deeper Inquiries