scholarly journals The Cockayne syndrome protein B is involved in the repair of 5-AZA-2′-deoxycytidine-induced DNA lesions

Oncotarget ◽  
2018 ◽  
Vol 9 (80) ◽  
pp. 35069-35084 ◽  
Author(s):  
Estefanía Burgos-Morón ◽  
José Manuel Calderón-Montaño ◽  
Nuria Pastor ◽  
Andreas Höglund ◽  
Ángel Ruiz-Castizo ◽  
...  
Keyword(s):  
Cockayne Syndrome ◽  
Dna Lesions ◽  
Syndrome Protein ◽  
Protein B

scholarly journals Reciprocally Regulated Chromatin Association of Cockayne Syndrome Protein B and p53 Protein

2011 ◽  
Vol 286 (40) ◽  
pp. 34951-34958 ◽  
Author(s):  
Robert J. Lake ◽  
Asjad Basheer ◽  
Hua-Ying Fan
Keyword(s):  
P53 Protein ◽  
Cockayne Syndrome ◽  
Syndrome Protein ◽  
Protein B

scholarly journals Cockayne syndrome protein B interacts with and is phosphorylated by c-Abl tyrosine kinase

2007 ◽  
Vol 35 (15) ◽  
pp. 4941-4951 ◽  
Author(s):  
S. Z. Imam ◽  
F. E. Indig ◽  
W.-H. Cheng ◽  
S. P. Saxena ◽  
T. Stevnsner ◽  
...  
Keyword(s):  
Tyrosine Kinase ◽  
Cockayne Syndrome ◽  
Abl Tyrosine Kinase ◽  
Syndrome Protein ◽  
Protein B

scholarly journals The Sequence-Specific Transcription Factor c-Jun Targets Cockayne Syndrome Protein B to Regulate Transcription and Chromatin Structure

PLoS Genetics ◽  
2014 ◽  
Vol 10 (4) ◽  
pp. e1004284 ◽  
Author(s):  
Robert J. Lake ◽  
Erica L. Boetefuer ◽  
Pei-Fang Tsai ◽  
Jieun Jeong ◽  
Inchan Choi ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Chromatin Structure ◽  
Cockayne Syndrome ◽  
Specific Transcription Factor ◽  
Factor C ◽  
Syndrome Protein ◽  
Protein B

scholarly journals Oxygen-Dependent Accumulation of Purine DNA Lesions in Cockayne Syndrome Cells

Cells ◽  
2020 ◽  
Vol 9 (7) ◽  
pp. 1671 ◽  
Author(s):  
Marios G. Krokidis ◽  
Mariarosaria D’Errico ◽  
Barbara Pascucci ◽  
Eleonora Parlanti ◽  
Annalisa Masi ◽  
...  
Keyword(s):  
Dna Damage ◽  
Oxidative Dna Damage ◽  
Excision Repair ◽  
Enzymatic Digestion ◽  
Cockayne Syndrome ◽  
Dna Lesions ◽  
Premature Aging ◽  
Neurological Features ◽  
Oxygen Tensions ◽  
Base Excision

Cockayne Syndrome (CS) is an autosomal recessive neurodegenerative premature aging disorder associated with defects in nucleotide excision repair (NER). Cells from CS patients, with mutations in CSA or CSB genes, present elevated levels of reactive oxygen species (ROS) and are defective in the repair of a variety of oxidatively generated DNA lesions. In this study, six purine lesions were ascertained in wild type (wt) CSA, defective CSA, wtCSB and defective CSB-transformed fibroblasts under different oxygen tensions (hyperoxic 21%, physioxic 5% and hypoxic 1%). In particular, the four 5′,8-cyclopurine (cPu) and the two 8-oxo-purine (8-oxo-Pu) lesions were accurately quantified by LC-MS/MS analysis using isotopomeric internal standards after an enzymatic digestion procedure. cPu levels were found comparable to 8-oxo-Pu in all cases (3–6 lesions/106 nucleotides), slightly increasing on going from hyperoxia to physioxia to hypoxia. Moreover, higher levels of four cPu were observed under hypoxia in both CSA and CSB-defective cells as compared to normal counterparts, along with a significant enhancement of 8-oxo-Pu. These findings revealed that exposure to different oxygen tensions induced oxidative DNA damage in CS cells, repairable by NER or base excision repair (BER) pathways. In NER-defective CS patients, these results support the hypothesis that the clinical neurological features might be connected to the accumulation of cPu. Moreover, the elimination of dysfunctional mitochondria in CS cells is associated with a reduction in the oxidative DNA damage.

scholarly journals Disruption of the Cockayne Syndrome B Gene Impairs Spontaneous Tumorigenesis in Cancer-Predisposed Ink4a/ARF Knockout Mice

2001 ◽  
Vol 21 (5) ◽  
pp. 1810-1818 ◽  
Author(s):  
Yi Lu ◽  
Hanzhou Lian ◽  
Prerna Sharma ◽  
Nicole Schreiber-Agus ◽  
Robert G. Russell ◽  
...  
Keyword(s):  
Dna Repair ◽  
Colony Formation ◽  
Gene Disruption ◽  
Formation Rate ◽  
Cockayne Syndrome ◽  
Dna Lesions ◽  
Synthesis Rate ◽  
Mrna Synthesis ◽  
Antineoplastic Effect ◽  
Repair Defect

ABSTRACT Cells isolated from individuals with Cockayne syndrome (CS) have a defect in transcription-coupled DNA repair, which rapidly corrects certain DNA lesions located on the transcribed strand of active genes. Despite this DNA repair defect, individuals with CS group A (CSA) or group B (CSB) do not exhibit an increased spontaneous or UV-induced cancer rate. In order to investigate the effect of CSB deficiency on spontaneous carcinogenesis, we crossed CSB−/− mice with cancer-prone mice lacking the p16Ink4a/p19ARFtumor suppressor locus. CSB−/− mice are sensitive to UV-induced skin cancer but show no increased rate of spontaneous cancer. CSB−/− Ink4a/ARF−/− mice developed 60% fewer tumors than Ink4a/ARF−/− animals and demonstrated a longer tumor-free latency time (260 versus 150 days). Moreover, CSB−/− Ink4a/ARF−/− mouse embryo fibroblasts (MEFs) exhibited a lower colony formation rate after low-density seeding, a lower rate of H-Ras-induced transformation, slower proliferation, and a lower mRNA synthesis rate than Ink4a/ARF−/− MEFs. CSB−/−Ink4a/ARF−/− MEFs were also more sensitive to UV-induced p53 induction and UV-induced apoptosis than were Ink4a/ARF−/− MEFs. In order to investigate whether the apparent antineoplastic effect of CSB gene disruption was caused by sensitization to genotoxin-induced (p53-mediated) apoptosis or by p53-independent sequelae, we also generated p53−/− and CSB−/− p53−/− MEFs. The CSB−/− p53−/− MEFs demonstrated lower colony formation efficiency, a lower proliferation rate, a lower mRNA synthesis rate, and a higher rate of UV-induced cell death than p53−/− MEFs. Collectively, these results indicate that the antineoplastic effect of CSB gene disruption is at least partially p53 independent; it may result from impaired transcription or from apoptosis secondary to environmental or endogenous DNA damage.

scholarly journals Repair protein persistence at DNA lesions characterizes XPF defect with Cockayne syndrome features

2018 ◽  
Vol 46 (18) ◽  
pp. 9563-9577 ◽  
Author(s):  
Mariangela Sabatella ◽  
Arjan F Theil ◽  
Cristina Ribeiro-Silva ◽  
Jana Slyskova ◽  
Karen Thijssen ◽  
...  
Keyword(s):  
Cockayne Syndrome ◽  
Dna Lesions ◽  
Repair Protein
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