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TranscriptomicsTranscription profiling of Cockayne Syndrome human fibroblasts lacking functional CSB, a SWI/SNF-like ATPase, to determine the molecular defect in CS
Cockayne syndrome (CS) is an inherited neurodevelopmental disorder with progeroid features. Although the genes responsible for CS have been implicated in a variety of DNA repair- and transcription-related pathways, the nature of the molecular defect in CS remains mysterious. We sought to define this...
ORGANISM(S): Homo sapiens 
2007-07-03 | E-GEOD-3407 | biostudies-arrayexpress
TranscriptomicsA High-Fat Diet and NAD+ Rescue Premature Aging in Cockayne Syndrome
Cockayne syndrome (CS) is an accelerated aging disorder characterized by progressive neurodegeneration caused by mutations in the genes encoding the DNA repair proteins CSA or CSB. Csbm/m mice were given a high-fat, caloric-restricted or resveratrol-supplemented diet. The high-fat diet rescued the p...
ORGANISM(S): Mus musculus 
2014-12-22 | E-GEOD-62194 | biostudies-arrayexpress
TranscriptomicsTranscriptional effects of CSB and the CSB-PGBD3 fusion protein in CSB-null UVSS1KO cells
Cockayne syndrome is a segmental progeria most often caused by mutations in the CSB gene encoding a SWI/SNF-like ATPase required for transcription-coupled DNA repair (TCR). Over 43 Mya before marmosets diverged from humans, a piggyBac3 (PGBD3) transposable element integrated into intron 5 of the CSB...
ORGANISM(S): Homo sapiens 
2014-03-20 | E-GEOD-56049 | biostudies-arrayexpress
ProteomicsRNA polymerase I transcription disturbances lead to instable ribosomes in Cockayne syndrome
DNA-repair factors of the Nucleotide -Excision-Repair (NER) pathway are part of the basal transcription apparatus of RNA polymerase I. Mutations in these factors can give rise to developmental disorders with symptoms that are typical for the aging body. Here we show that in Cockayne syndrome (CS) RN...
ORGANISM(S): Homo sapiens (Human) 
2021-11-16 | PXD024478 | Pride
Cockayne syndrome Translational fidelity Ribosome Loss of proteostasis Rna polymerase i 
TranscriptomicsRegulatory interplay of Cockayne syndrome B ATPase and stress-response gene ATF3 following genotoxic stress
Gene expression profile in CS1AN deficient and CSBwt restored cell lines after 24 hours of UV or alphe-amanitin treatment (only for restored). The comaprison of expression profile between 0 and 24 hours revealed continouse suppresion of transcription upon UV treatment in CS1AN cell line and alpha-am...
ORGANISM(S): Homo sapiens 
2014-05-23 | E-GEOD-57923 | biostudies-arrayexpress
Genomicsc-Jun Targets Cockayne Syndrome protein B to Specific Genomic Region for Transcription Regulation
Cockayne syndrome is an inherited premature aging syndrome associated with developmental and neurological disorders. Mutations in the genomic locus encoding CSB are associated with 80% Cockayne syndrome cases. Transcription profiling assays reveal the association of mis-regulation of gene expressi...
ORGANISM(S): Homo sapiens 
2014-03-06 | E-GEOD-50171 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of liver from Csbm/m/Xpa-/- knockout mice to investigate the effect of nucleotide excision repair pathway inactivation
Cockayne syndrome (CS) is a photosensitive, DNA repair disorder associated with progeria caused by a defect in the transcription-coupled repair (TCR) subpathway of nucleotide excision repair (NER). Here, complete inactivation of NER in Csbm/m/Xpa-/- mutants causes a phenotype that reliably mimics th...
ORGANISM(S): Mus musculus 
2007-01-01 | E-MEXP-835 | biostudies-arrayexpress
GenomicsGenome wide profiling of RNA Pol II and CSB in CS1AN cells or wild type CSB reconstituted cells [ChIP-seq]
We report how CSB affects globally the density of RNA Pol II at TSS and how this effect correlates with the gene expression alterations observed from microarray analysis. We also show globally the distribution of CSB. RNA Pol II and CSB ChIP-Seq in CS1AN cells and CSB reconstituted wild type cells, ...
ORGANISM(S): Homo sapiens 
2014-09-22 | E-GEOD-58253 | biostudies-arrayexpress
TranscriptomicsExpression data in induced pluripotent stem cells (iPSCs) derived from a DNA repair deficient fibroblast
Cockayne syndrome (CS) is an autossomal human disorder characterized by premature aging along with other symptoms. At the molecular level, CS is characterized by a deficiency in the Transcription-couple DNA repair pathway caused by a mutation mainly in ERCC6 gene and the absence of its functional pr...
ORGANISM(S): Homo sapiens 
2012-07-12 | E-GEOD-36648 | biostudies-arrayexpress
TranscriptomicsTranscription profiling of lung tissue from transcription-coupled repair-deficient (Csb-/-) and wild type mice in response to ozone
We investigated whether transcription-coupled repair deficient mice (Cockayne syndrome B knockout mice (Csb-/-)), known to be sensitive to oxidative stressors, have a different response to ozone than its repair-proficient control, Csb heterozygote (Csb+/-) mice.
ORGANISM(S): Mus musculus 
2006-11-13 | E-TABM-137 | biostudies-arrayexpress
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