Cockayne syndrome group B protein has novel strand annealing and exchange activities
نویسندگان
چکیده
منابع مشابه
Cockayne syndrome group B protein has novel strand annealing and exchange activities
Cockayne syndrome (CS) is a rare inherited human genetic disorder characterized by UV sensitivity, severe neurological abnormalities and prageroid symptoms. The CS complementation group B (CSB) protein is involved in UV-induced transcription coupled repair (TCR), base excision repair and general transcription. CSB also has a DNA-dependent ATPase activity that may play a role in remodeling chrom...
متن کاملCockayne syndrome group B protein regulates DNA double-strand break repair and checkpoint activation.
Mutations of CSB account for the majority of Cockayne syndrome (CS), a devastating hereditary disorder characterized by physical impairment, neurological degeneration and segmental premature aging. Here we report the generation of a human CSB-knockout cell line. We find that CSB facilitates HR and represses NHEJ. Loss of CSB or a CS-associated CSB mutation abrogating its ATPase activity impairs...
متن کاملCockayne syndrome group B protein (CSB) plays a general role in chromatin maintenance and remodeling.
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. Using expression microarrays and a unique method for comparative expression analysis called L2L, we sought to define this...
متن کاملCockayne Syndrome group B protein interacts with TRF2 and regulates telomere length and stability
The majority of Cockayne syndrome (CS) patients carry a mutation in Cockayne Syndrome group B (CSB), a large nuclear protein implicated in DNA repair, transcription and chromatin remodeling. However, whether CSB may play a role in telomere metabolism has not yet been characterized. Here, we report that CSB physically interacts with TRF2, a duplex telomeric DNA binding protein essential for telo...
متن کاملMolecular characterization of an acidic region deletion mutant of Cockayne syndrome group B protein.
Cockayne syndrome (CS) is a human genetic disorder characterized by post-natal growth failure, neurological abnormalities and premature aging. CS cells exhibit high sensitivity to UV light, delayed RNA synthesis recovery after UV irradiation and defective transcription-coupled repair (TCR). Two genetic complementation groups of CS have been identified, designated CS-A and CS-B. The CSB gene enc...
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ژورنال
عنوان ژورنال: Nucleic Acids Research
سال: 2006
ISSN: 0305-1048,1362-4962
DOI: 10.1093/nar/gkj410