Journal
MECHANISMS OF AGEING AND DEVELOPMENT
Volume 134, Issue 5-6, Pages 212-224Publisher
ELSEVIER IRELAND LTD
DOI: 10.1016/j.mad.2013.03.009
Keywords
Cockayne syndrome; Protein interactions; DNA repair deficiency; Transcription deficiency; Mitochondria
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Funding
- Intramural Program at the National Institutes on Aging, National Institutes of Health
- Velux Foundation
- NovoNordic Foundation
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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. (c) 2013 Elsevier Ireland Ltd. All rights reserved.
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