Article
Loss of Proteostasis Is a Pathomechanism in Cockayne Syndrome.
Cell reports - 8 May 2018
Alupei Marius Costel, Maity Pallab, Esser Philipp Ralf, Krikki Ioanna, Tuorto Francesca, Parlato Rosanna, Penzo Marianna, Schelling Adrian, Laugel Vincent, Montanaro Lorenzo, Scharffetter-Kochanek Karin, Iben Sebastian
Abstract excerpt
Retarded growth and neurodegeneration are hallmarks of the premature aging disease Cockayne syndrome (CS). Cockayne syndrome proteins take part in the key step of ribosomal biogenesis, transcription of RNA polymerase I. Here, we identify a mechanism originating from a disturbed RNA polymerase I transcription that impacts translational fidelity of the ribosomes and consequently produces misfolded proteins. In...
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