Multiple interaction partners for Cockayne syndrome proteins: implications for genome and transcriptome maintenance.

Abstract

Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.

• Authors: Maria D Aamann, Meltem Muftuoglu, Vilhelm A Bohr, Tinna Stevnsner
• Journal: Mechanisms of ageing and development (Mech Ageing Dev) 2013 May-Jun Vol. 134 Issue 5-6 Pg. 212-24 ISSN: 1872-6216 [Electronic] Ireland
• PMID: 23583689 (Publication Type: Journal Article, Research Support, N.I.H., Intramural, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Chemical References

• ERCC8 protein, human
• Poly-ADP-Ribose Binding Proteins
• Transcription Factors
• DNA Helicases
• ERCC6 protein, human
• DNA Repair Enzymes

Topics

• Animals
• DNA Damage
• DNA Helicases (genetics, metabolism)
• DNA Repair
• DNA Repair Enzymes (genetics, metabolism)
• Genome, Human
• Humans
• Poly-ADP-Ribose Binding Proteins
• Transcription Factors (genetics, metabolism)
• Transcriptome