Molecular characterization of an acidic region deletion mutant of Cockayne syndrome group B protein
نویسندگان
چکیده
منابع مشابه
Molecular 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...
متن کاملThe ATPase domain but not the acidic region of Cockayne syndrome group B gene product is essential for DNA repair.
Cockayne syndrome (CS) is a human genetic disorder characterized by UV sensitivity, developmental abnormalities, and premature aging. Two of the genes involved, CSA and CSB, are required for transcription-coupled repair (TCR), a subpathway of nucleotide excision repair that removes certain lesions rapidly and efficiently from the transcribed strand of active genes. CS proteins have also been im...
متن کاملThe role of Cockayne Syndrome Protein B in transcription regulation
We investigated the question if CSB (Cockayne Syndrome complementation B) protein actively regulates gene transcription and how mutations in CSB gene affect that regulatory role. Here we describe how we processed and interpreted ChIP-seq data (deposited in Gene Expression Omnibus with accession number GSE50171) obtained during an investigation of that question, and how this analysis assisted in...
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Cockayne syndrome (CS) is a devastating autosomal recessive disease characterized by neurodegeneration, cachexia, and accelerated aging. 80% of the cases are caused by mutations in the CS complementation group B (CSB) gene known to be involved in DNA repair and transcription. Recent evidence indicates that CSB is present in mitochondria, where it associates with mitochondrial DNA (mtDNA). We re...
متن کاملBiochemical and biological characterization of wild-type and ATPase-deficient Cockayne syndrome B repair protein.
Cockayne syndrome (CS) is a nucleotide excision repair disorder characterized by sun (UV) sensitivity and severe developmental problems. Two genes have been shown to be involved: CSA and CSB. Both proteins play an essential role in preferential repair of transcription-blocking lesions from active genes. In this study we report the purification and characterization of baculovirus-produced HA-His...
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ژورنال
عنوان ژورنال: Nucleic Acids Research
سال: 2000
ISSN: 1362-4962
DOI: 10.1093/nar/28.16.3151