Cockayne Syndrome-Associated CSA and CSB Mutations Impair Ribosome Biogenesis, Ribosomal Protein Stability, and Global Protein Folding

Cockayne Syndrome-Associated CSA and CSB Mutations Impair Ribosome Biogenesis, Ribosomal Protein Stability, and Global Protein Folding

Cockayne syndrome (CS) is a developmental disorder with symptoms that are typical for the aging body, including subcutaneous fat loss, alopecia, and cataracts. Here, we show that in the cells of CS patients, RNA polymerase I transcription and the processing of the pre-rRNA are disturbed, leading to...

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Bibliographic Details
Main Authors: Mingyue Qiang, Fatima Khalid, Tamara Phan, Christina Ludwig, Karin Scharffetter-Kochanek, Sebastian Iben
Format: Article
Language:English
Published: MDPI AG 2021-06-01
Series:Cells
Subjects:
RNA polymerase I
ribosome
Cockayne syndrome
translational fidelity
loss of proteostasis
Online Access:https://www.mdpi.com/2073-4409/10/7/1616

Internet

https://www.mdpi.com/2073-4409/10/7/1616

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