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

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  • Maria D Aamann, Denmark
  • Meltem Muftuoglu, Koc University, School of Medicine, Department of Biochemistry, Turkey
  • Vilhelm A Bohr, Laboratory of Molecular Gerontology, National Institute on Aging, National Institutes of Healt, United States
  • Tinna Stevnsner
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.
Original languageEnglish
JournalMechanisms of Ageing and Development
Pages (from-to)212-224
Number of pages13
Publication statusPublished - May 2013

    Research areas

  • Animals, DNA Damage, DNA Helicases, DNA Repair, DNA Repair Enzymes, Genome, Human, Humans, Transcription Factors, Transcriptome

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