CSB is a component of RNA pol I transcription
| Autor: | Luca Proietti De Santis, Jérôme Auriol, John Bradsher, Ingrid Grummt, Jean-Marc Egly, Sebastian Iben, Jean Luc Vonesch |
|---|---|
| Rok vydání: | 2002 |
| Předmět: |
musculoskeletal diseases
congenital hereditary and neonatal diseases and abnormalities Transcription Genetic Nucleolus Blotting Western Fluorescent Antibody Technique Biology Transcription Factors TFII Transcription (biology) Multienzyme Complexes RNA Polymerase I RNA polymerase I Humans Poly-ADP-Ribose Binding Proteins Gene Molecular Biology Cells Cultured Xeroderma Pigmentosum Group D Protein Genetics Nucleoplasm DNA Helicases nutritional and metabolic diseases RNA Nuclear Proteins Proteins Transfection Cell Biology Fibroblasts Endonucleases Cell biology DNA-Binding Proteins DNA Repair Enzymes RNA Ribosomal Mutation Transcription factor II H Transcription Factor TFIIH Cell Nucleolus Protein Binding Transcription Factors |
| Zdroj: | Molecular cell. 10(4) |
| ISSN: | 1097-2765 |
| Popis: | Mutation in the CSB gene results in the human Cockayne's syndrome (CS). Here, we provide evidence that CSB is found not only in the nucleoplasm but also in the nucleolus within a complex (CSB IP/150) that contains RNA pol I, TFIIH, and XPG and promotes efficient rRNA synthesis. CSB is active in in vitro RNA pol I transcription and restores rRNA synthesis when transfected in CSB-deficient cells. We also show that mutations in CSB, as well as in XPB and XPD genes, all of which confer CS, disturb the RNA pol I/TFIIH interaction within the CSB IP/150. In addition to revealing an unanticipated function for CSB in rRNA synthesis, we show that the fragility of this complex could be one factor contributing to the CS phenotype. |
| Databáze: | OpenAIRE |
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