Cockayne Syndrome Group B (CSB): The Regulatory Framework Governing the Multifunctional Protein and Its Plausible Role in Cancer
Zoi Spyropoulou,
Angelos Papaspyropoulos,
Nefeli Lagopati,
Vassilios Myrianthopoulos,
Alexandros G. Georgakilas,
Maria Fousteri,
Athanassios Kotsinas,
Vassilis G. Gorgoulis
Affiliations
Zoi Spyropoulou
Biomedical Sciences Research Center Alexander Fleming, Institute for Fundamental Biomedical Research, 16672 Athens, Greece
Angelos Papaspyropoulos
Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece
Nefeli Lagopati
Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece
Vassilios Myrianthopoulos
Department of Pharmacy, National and Kapodistrian University of Athens, 15771 Athens, Greece
Alexandros G. Georgakilas
DNA Damage Laboratory, Physics Department, School of Applied Mathematical and Physical Sciences, National Technical University of Athens (NTUA), 15780 Athens, Greece
Maria Fousteri
Biomedical Sciences Research Center Alexander Fleming, Institute for Fundamental Biomedical Research, 16672 Athens, Greece
Athanassios Kotsinas
Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece
Vassilis G. Gorgoulis
Molecular Carcinogenesis Group, Department of Histology and Embryology, School of Medicine, National and Kapodistrian University of Athens, 11527 Athens, Greece
Cockayne syndrome (CS) is a DNA repair syndrome characterized by a broad spectrum of clinical manifestations such as neurodegeneration, premature aging, developmental impairment, photosensitivity and other symptoms. Mutations in Cockayne syndrome protein B (CSB) are present in the vast majority of CS patients and in other DNA repair-related pathologies. In the literature, the role of CSB in different DNA repair pathways has been highlighted, however, new CSB functions have been identified in DNA transcription, mitochondrial biology, telomere maintenance and p53 regulation. Herein, we present an overview of identified structural elements and processes that impact on CSB activity and its post-translational modifications, known to balance the different roles of the protein not only during normal conditions but most importantly in stress situations. Moreover, since CSB has been found to be overexpressed in a number of different tumors, its role in cancer is presented and possible therapeutic targeting is discussed.
