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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 
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 
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 
The CSB-PGBD3 fusion protein arose over 43 million years ago when a 2.5 kb piggyBac 3 (PGBD3) transposon inserted into intron 5 of the Cockayne syndrome Group B (CSB) gene in the common ancestor of all higher primates. The CSB-PGBD3 fusion protein binds internally-deleted PGBD3 elements called MER85...
ORGANISM(S): Homo sapiens 
PxP-MS (Purification of x-linked Proteins coupled to Mass Spectrometry) was used to assess the role of CSB in DNA-protein crosslink repair. The CSB protein is a sensor that can detect stalled RNA polymerases at sites of DNA damage, thereby triggering transcription-coupled repair mechanisms. DPCs wer...
ORGANISM(S): Homo sapiens (Human) 
2024-02-15 | PXD047668 | Pride
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 
Transcription-Coupled Nucleotide Excision Repair (TC-NER) preserves transcriptional integrity by repairing transcription-blocking lesions (TBLs), which are highly cytotoxic if left unresolved. TC-NER is initiated when RNA Polymerase II (Pol II) stalls at a lesion and is recognized by CSB, followed b...
ORGANISM(S): Homo sapiens (Human) 
2026-06-18 | PXD074100 | Pride
The coordinated transcription of genes involves RNA polymerase II enzymes (RNAPII), which pull DNA through their active sites. DNA lesions in transcribed strands block RNAPII elongation and induce a strong transcriptional arrest. The transcription-coupled repair (TCR) pathway ensures the efficient r...
ORGANISM(S): Homo sapiens (Human) 
2021-02-28 | PXD016198 | Pride
The response to DNA damage-stalled RNA polymerase II (RNAPIIo) involves the assembly of the transcription-coupled repair (TCR) complex on actively transcribed strands. The function of the TCR proteins CSB, CSA and UVSSA and the manner in which the core DNA repair complex, including transcription fac...
ORGANISM(S): Homo sapiens (Human) 
2020-04-14 | PXD017329 | Pride
CSB-depletion induced SH-SY5Y differentiation defects can be partially rescued by re-expression of SYT9 gene. This study characterizes the transcriptome signatures upon SYT9 re-expression in CSB-KD SH-SY5Y cells after RA treatment. The Nimblegen human 12 x 135K gene expression array was used to char...
ORGANISM(S): Homo sapiens 
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