DNA Damage Responses and Oxidative Stress in Dyskeratosis Congenita

• Published in: PloS one Vol. 8; no. 10; p. e76473
• Main Authors: Pereboeva, Larisa, Westin, Erik, Patel, Toral, Flaniken, Ian, Lamb, Lawrence, Klingelhutz, Aloysius, Goldman, Frederick
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 04.10.2013; Public Library of Science (PLoS)
• Subjects: Acetylcysteine; Aging; Anemia; Antineoplastic Agents - toxicity; Antioxidants; Antioxidants (Nutrients); Apoptosis; Apoptosis - drug effects; Apoptosis - genetics; Apoptosis - radiation effects; Biomarkers; Bone cancer; Bone marrow; Bone marrow transplantation; Cancer; Cell culture; Cell cycle; Cell growth; Children & youth; Consent; Cysteine; Cystine; Cytometry; Cytotoxic agents; Cytotoxicity; Damage assessment; Deoxyribonucleic acid; DNA; DNA Damage; DNA repair; Dyskeratosis; Dyskeratosis Congenita - genetics; Dyskeratosis Congenita - metabolism; Etoposide; Exhaustion; Fibroblasts; Flow cytometry; Gene expression; Gene Expression Regulation - drug effects; Gene Expression Regulation - radiation effects; Genetic aspects; Hematology; Hospitals; Humans; Immunological tolerance; Ionizing radiation; Lymphocytes; Lymphocytes T; Medical research; Mutation; Oncology; Oxidative Stress; Oxidizing agents; Oxygen; p53 Protein; Paclitaxel; Pathogenesis; Pathology; Pediatrics; Pharmacology; Phenotypes; Proteins; Radiation; Radiation effects; Radiation therapy; Reactive oxygen species; Reactive Oxygen Species - metabolism; Review boards; Senescence; Stem cells; T cells; T-Lymphocytes - drug effects; T-Lymphocytes - metabolism; T-Lymphocytes - radiation effects; Telomerase; Telomeres; Toxicity; Transplantation; Transplants & implants; Tumor proteins; Tumor Suppressor Protein p53 - genetics; Western blotting; Alabama; United States > US
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0076473

Dyskeratosis congenita (DC) is an inherited multisystem disorder of premature aging, cancer predisposition, and bone marrow failure caused by selective exhaustion of highly proliferative cell pools. DC patients also have a poor tolerance to chemo/radiotherapy and bone marrow transplantation. Althoug...