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Study found that ERCC6 transcription may be epigenetically regulated in lens epithelial cells of age-related nuclear cataract leading to its repression.
Mutation of Cockayne syndrome B (CSB) affects neuronal gene expression and differentiation, so we attempted to bypass its function by expressing downstream target genes.
The frequency of the XPD (show ERCC2 Proteins) 312Asn allele was significantly higher in T >/= 2 high grade than in T >/= 2 low grade tumors (p = 0.036); the ERCC6 1097Val/Val genotype was strongly associated with muscle-invasive tumors.
new role of VCP/p97 (show vcp Proteins) segregase in the timely processing of ubiquitinated CSB from damaged chromatin.
CSB and CTCF (show CTCF Proteins) can regulate each other's chromatin association, thereby modulating chromatin structure and coordinating gene expression in response to oxidative stress.
Transcription inhibition reduced accumulation of CSB at sites of monoadducts and interstrand crosslinks, but it did not affect recruitment to (although slightly affected retention at) oxidative DNA damage.
Data suggest that both the most C-terminal region and SUMOylation of a lysine residue in N-terminal region are important for functions of CSB/ERCC6 in transcription-coupled nucleotide excision repair following DNA breakage from UV light.
ERCC6 dysfunction presenting as progressive neurological decline with brain hypomyelination. This report expands the disease spectrum associated with ERCC6 mutations.
33 proteins that were not previously known to interact with CSB.
Data indicate that Cockayne syndrome group B protein CSB function is necessary for the recruitment of recombinational factors.
Our data show that in addition to promoting repeat expansion, CSB does in fact protect the genome from germ line expansions in the FXD mouse model.
loss of CSB affects tandem-repeat expansions in a gender and cell-type-specific manner
CSB has been shown to regulate processes such as the transcriptional recovery after DNA damage, the p53 (show TP53 Proteins) transcriptional response, the response to hypoxia, the response IGF-1 (show IGF1 Proteins), transactivation of nuclear receptors, transcription of housekeeping genes
CSB and PCAF (show KAT2B Proteins) play cooperative roles to establish the active state of rRNA genes by histone acetylation
Csb-/- neural precursors exhibited defective self-renewal in the neurosphere assay.
Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a cs-b xp-c murine model of Cockayne syndrome
Testicular nuclear receptor 4 (TR4 (show NR2C2 Proteins)) regulates UV light-induced responses via Cockayne syndrome B protein-mediated transcription-coupled DNA repair
Data suggest that Cockayne syndrome B protein protects CAG repeats from expansion by either active reduction of the tract length during parent-child transmission, or by antagonizing the action of OGG1 (show OGG1 Proteins), which tends to promote expansion in somatic cells.
These results suggested that reduced repair of a DNA topoisomerase I-DNA covalent complex because of truncated CSB proteins is involved in the pathogenesis of CS-B.
MtDNA mutations are highly increased in cells from subcutaneous fat of aged Csb(m/m) mice
This gene encodes a DNA-binding protein that is important in transcription-coupled excision repair. The encoded protein has ATP-stimulated ATPase activity, interacts with several transcription and excision repair proteins, and may promote complex formation at DNA repair sites. Mutations in this gene are associated with Cockayne syndrome type B and cerebrooculofacioskeletal syndrome 1. Naturally-occurring readthrough transcription occurs between this gene and the adjacent PGBD3 gene (GeneID:267004), and results in a fusion protein that shares sequence with the product of each individual gene. The readthrough locus is represented by GeneID:101243544.
ATP-dependent helicase ERCC6
, Cockayne syndrome group B protein
, DNA excision repair protein ERCC-6
, cockayne syndrome protein CSB
, CS group B correcting
, excision repair cross-complementing rodent repair deficiency, complementation group 6