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

Maria D Aamann, Meltem Muftuoglu, Vilhelm A Bohr, Tinna Stevnsner

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    30 Citations (Scopus)

    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.
    Original languageEnglish
    JournalMechanisms of Ageing and Development
    Volume134
    Issue5-6
    Pages (from-to)212-224
    Number of pages13
    ISSN0047-6374
    DOIs
    Publication statusPublished - May 2013

    Keywords

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

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