Τίτλος:
Cockayne syndrome group B (CSB): The regulatory framework governing the multifunctional protein and its plausible role in cancer
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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. © 2021 by the authors. Licensee MDPI, Basel, Switzerland.
Συγγραφείς:
Spyropoulou, Z.
Papaspyropoulos, A.
Lagopati, N.
Myrianthopoulos, V.
Georgakilas, A.G.
Fousteri, M.
Kotsinas, A.
Gorgoulis, V.G.
Περιοδικό:
Cell Stem Cell
Λέξεις-κλειδιά:
adenosine triphosphate; BRCA1 protein; cisplatin; deubiquitinase; hypoxia inducible factor 1alpha; nicotinamide adenine dinucleotide adenosine diphosphate ribosyltransferase 1; protein p53; pyrimidine dimer; RNA polymerase II; ubiquitin carboxyl terminal hydrolase 7; ubiquitin protein ligase E3; vasculotropin; DNA helicase; DNA ligase; ERCC6 protein, human; poly ADP ribose binding protein, Caenorhabditis elegans; cancer cell; cancer susceptibility; cancer therapy; cell cycle arrest; cell proliferation; cerebellum atrophy; Cockayne syndrome; COFS syndrome; CRISPR-CAS9 system; DNA damage; DNA repair; DNA transcription; endoplasmic reticulum stress; ERCC6 gene; gene; gene mutation; gene overexpression; genotype; lipid peroxidation; nerve degeneration; nonhuman; pathology; phosphorylation; photosensitivity; premature aging; progression free survival; protein processing; Review; RNA interference; single nucleotide polymorphism; site directed mutagenesis; sumoylation; telomere; trichothiodystrophy; ubiquitination; ultraviolet irradiation; upregulation; animal; chemistry; Cockayne syndrome; gene expression regulation; genetics; human; metabolism; molecular model; mutation; neoplasm; protein conformation, Animals; Cockayne Syndrome; DNA Damage; DNA Helicases; DNA Repair; DNA Repair Enzymes; Gene Expression Regulation, Neoplastic; Humans; Models, Molecular; Mutation; Neoplasms; Poly-ADP-Ribose Binding Proteins; Protein Conformation; Protein Processing, Post-Translational
DOI:
10.3390/cells10040866
