A Ubiquitin-Binding Domain in Cockayne Syndrome B Required for Transcription-Coupled Nucleotide Excision Repair
| Autor: | Roy Anindya, Pierre-Olivier Mari, Wim Vermeulen, Giuseppina Giglia-Mari, Maria Fousteri, Jean-Marc Egly, Jesper Q. Svejstrup, Leon H.F. Mullenders, Ulrik Kristensen, Hanneke Kool |
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| Přispěvatelé: | Université Paris 1 Panthéon-Sorbonne - UFR d'Arts plastiques et sciences de l'art (UP1 UFR04), Université Paris 1 Panthéon-Sorbonne (UP1), Institut de génétique et biologie moléculaire et cellulaire (IGBMC), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Louis Pasteur - Strasbourg I, The Francis Crick Institute [London], Molecular Genetics |
| Rok vydání: | 2010 |
| Předmět: |
musculoskeletal diseases
congenital hereditary and neonatal diseases and abnormalities Ubiquitin binding DNA Repair DNA repair Ultraviolet Rays Recombinant Fusion Proteins Molecular Sequence Data RNA polymerase II medicine.disease_cause Cockayne syndrome Cell Line 03 medical and health sciences 0302 clinical medicine Ubiquitin medicine Humans Poly-ADP-Ribose Binding Proteins Amino Acid Sequence Cockayne Syndrome Promoter Regions Genetic Molecular Biology ComputingMilieux_MISCELLANEOUS 030304 developmental biology Cell Nucleus 0303 health sciences Mutation biology DNA Helicases nutritional and metabolic diseases [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry Molecular Biology/Molecular biology Cell Biology medicine.disease Molecular biology Protein Structure Tertiary Tetrahydrofolate Dehydrogenase DNA Repair Enzymes rna-polymerase-ii induced dna-damage xeroderma-pigmentosum uv-irradiation active genes cells complexes protein ubiquitylation recognition biology.protein RNA Polymerase II Sequence Alignment 030217 neurology & neurosurgery Nucleotide excision repair DNA Damage |
| Zdroj: | Molecular Cell Molecular Cell, Elsevier, 2010, 38 (5), pp.637-648. ⟨10.1016/j.molcel.2010.04.017⟩ Molecular Cell, 38(5), 637-648. Cell Press Molecular Cell, 38(5), 637-648 |
| ISSN: | 1097-4164 1097-2765 |
| Popis: | Transcription-coupled nucleotide excision repair (TC-NER) allows RNA polymerase II (RNAPII)-blocking lesions to be rapidly removed from the transcribed strand of active genes. Defective TCR in humans is associated with Cockayne syndrome (CS), typically caused by defects in either CSA or CSB. Here, we show that CSB contains a ubiquitin-binding domain (UBD). Cells expressing UBD-less CSB (CSB(del)) have phenotypes similar to those of cells lacking CSB, but these can be suppressed by appending a heterologous UBD, so ubiquitin binding is essential for CSB function. Surprisingly, CSB(del) remains capable of assembling nucleotide excision repair factors and repair synthesis proteins around damage-stalled RNAPII, but such repair complexes fail to excise the lesion. Together, our results indicate an essential role for protein ubiquitylation and CSB's UBD in triggering damage incision during TC-NER and allow us to integrate the function of CSA and CSB in a model for the process. |
| Databáze: | OpenAIRE |
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