Cockayne syndrome, underlying molecular defects and p53

Abstract

Comment on: Latini P, et al. Cell Cycle 2011; 10:3719-30.

Figures and Tables

Figure 1 Molecular roles of CS proteins. CSA and CSB operate in DNA repair as components of the TC-NER pathway which removes RNA polymerase blocking lesions and in repair of endogenous DNA damage in the nucleus and mitochondria by interacting with BER/SSBR proteins and stimulating removal of base damage. CSB acts in basal nuclear transcription by promoting elongation by RNAPI and RNAPII and plays a larger overall role in transcriptome regulation/programming. CSA and CSB both physically interact with p53 and stimulate Mdm2-dependent ubiquitination and subsequent degradation of p53. In the absence of CSA or CSB, Mdm2 ubiquitination of p53 is lower, stabilizing p53 and promoting pro-apoptotic signals through p53 dependent transcriptional programming.