Transcription-coupled DNA repair: from mechanism to human disorder

D van den Heuvel, Y van der Weegen, DEC Boer… - Trends in Cell …, 2021 - cell.com
DNA lesions pose a major obstacle during gene transcription by RNA polymerase II (RNAPII)
enzymes. The transcription-coupled DNA repair (TCR) pathway eliminates such DNA …

[HTML][HTML] The cooperative action of CSB, CSA, and UVSSA target TFIIH to DNA damage-stalled RNA polymerase II

Y van der Weegen, H Golan-Berman… - Nature …, 2020 - nature.com
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 …

ELOF1 is a transcription-coupled DNA repair factor that directs RNA polymerase II ubiquitylation

Y Van der Weegen, K de Lint, D van den Heuvel… - Nature cell …, 2021 - nature.com
Cells employ transcription-coupled repair (TCR) to eliminate transcription-blocking DNA
lesions. DNA damage-induced binding of the TCR-specific repair factor CSB to RNA …

[HTML][HTML] A CSB-PAF1C axis restores processive transcription elongation after DNA damage repair

…, D Zhou, H Wu, K Apelt, Y van der Weegen… - Nature …, 2021 - nature.com
Bulky DNA lesions in transcribed strands block RNA polymerase II (RNAPII) elongation and
induce a genome-wide transcriptional arrest. The transcription-coupled repair (TCR) …

[HTML][HTML] A comparative study of SMN protein and mRNA in blood and fibroblasts in patients with spinal muscular atrophy and healthy controls

RI Wadman, M Stam, MD Jansen, Y van der Weegen… - PloS one, 2016 - journals.plos.org
Background Clinical trials to test safety and efficacy of drugs for patients with spinal
muscular atrophy (SMA) are currently underway. Biomarkers that document treatment-induced …

[HTML][HTML] Structural basis for RNA polymerase II ubiquitylation and inactivation in transcription-coupled repair

…, PJ van der Meer, Y van der Weegen… - Nature Structural & …, 2024 - nature.com
During transcription-coupled DNA repair (TCR), RNA polymerase II (Pol II) transitions from a
transcriptionally active state to an arrested state that allows for removal of DNA lesions. This …

[HTML][HTML] TRiC controls transcription resumption after UV damage by regulating Cockayne syndrome protein A

…, MG Vrouwe, Y Van Der Weegen… - Nature …, 2018 - nature.com
Transcription-blocking DNA lesions are removed by transcription-coupled nucleotide
excision repair (TC-NER) to preserve cell viability. TC-NER is triggered by the stalling of RNA …

Transcription-coupled nucleotide excision repair is coordinated by ubiquitin and SUMO in response to ultraviolet irradiation

…, ME van Royen, Y van der Weegen… - Nucleic Acids …, 2020 - academic.oup.com
Cockayne Syndrome (CS) is a severe neurodegenerative and premature aging autosomal-recessive
disease, caused by inherited defects in the CSA and CSB genes, leading to defects …

[HTML][HTML] Mono-ubiquitination of Rabphilin 3A by UBE3A serves a non-degradative function

…, FI Zampeta, IJ de Graaf, Y van der Weegen… - Scientific reports, 2021 - nature.com
Angelman syndrome (AS) is a severe neurodevelopmental disorder caused by brain-specific
loss of UBE3A, an E3 ubiquitin protein ligase. A substantial number of possible …

The sequential and cooperative action of CSB, CSA and UVSSA targets the TFIIH complex to DNA damage-stalled RNA polymerase II

Y van der Weegen, HG Berman, TET Mevissen, K Apelt… - bioRxiv, 2019 - biorxiv.org
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 …