Loss of Proteostasis Is a Pathomechanism in Cockayne Syndrome

Summary: 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 polymera...

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Bibliographic Details
Main Authors: Marius Costel Alupei, Pallab Maity, Philipp Ralf Esser, Ioanna Krikki, Francesca Tuorto, Rosanna Parlato, Marianna Penzo, Adrian Schelling, Vincent Laugel, Lorenzo Montanaro, Karin Scharffetter-Kochanek, Sebastian Iben
Format: Article
Language:English
Published: Elsevier 2018-05-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124718305850