CSB is a component of RNA pol I transcription

John Bradsher , Jerome Auriol , Luca Proietti de Santis , Sebastian Iben , Jean Luc Vonesch , Ingrid Grummt , Jean-Marc Egly (1, 2)

1 IGBMC - Institut de génétique et biologie moléculaire et cellulaire
2 IGBMC - Institut de Génétique et de Biologie Moléculaire et Cellulaire

Mutation in the CSB gene results in the human Cockayne's syndrome (CS). Here, we provide evidence that CSB is found not only in the nucleoplasm but also in the nucleolus within a complex (CSB IP/150) that contains RNA pol I, TFIIH, and XPG and promotes efficient rRNA synthesis. CSB is active in in vitro RNA pol I transcription and restores rRNA synthesis when transfected in CSB-deficient cells. We also show that mutations in CSB, as well as in XPB and XPD genes, all of which confer CS, disturb the RNA pol I/TFIIH interaction within the CSB IP/150. In addition to revealing an unanticipated function for CSB in rRNA synthesis, we show that the fragility of this complex could be one factor contributing to the CS phenotype.

Domaines: Biochimie, Biologie Moléculaire

John Bradsher, Jerome Auriol, Luca Proietti de Santis, Sebastian Iben, Jean Luc Vonesch, et al.. CSB is a component of RNA pol I transcription. Molecular Cell, 2002, 10 (4), pp.819-829. ⟨10.1016/s1097-2765(02)00678-0⟩. ⟨hal-04135462⟩