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anti-Human ERCC6 Antikörper:
anti-Rat (Rattus) ERCC6 Antikörper:
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Gene analysis showed mutations in exons 4 and 18 of the ERCC6 gene. Multiple ocular abnormalities were observed in a patient with Cockayne syndrome. A detailed ophthalmic evaluation of children with Cockayne syndrome is advised.
The role of ERCC6 in regulating response to 5-fluorouracil and drug resistance in colorectal cancer.Elevated expression of ERCC6 is associated with poor colorectal cancer patient survival.
NAP1L1 (zeige NAP1L1 Antikörper) increases CSB processivity by decreasing the pausing probability during translocation. Our study, therefore, uncovers the different steps of CSB-mediated chromatin remodeling that can be regulated by NAP1L1 (zeige NAP1L1 Antikörper).
pro-apoptotic effects observed after CSB ablation
the Elongin A ubiquitin ligase and the CSB protein function together in a common pathway in response to Pol II stalling and DNA damage
No significant association exists between ERCC6 polymorphisms and bladder cancer risk.
CSB plays a role in the homeostasis and function of human neurons. CSB-deficient neural networks displayed altered electrophysiological activity, including decreased synchrony, and reduced synapse density.
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 (zeige ERCC2 Antikörper) 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.
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 (zeige TP53 Antikörper) transcriptional response, the response to hypoxia, the response IGF-1 (zeige IGF1 Antikörper), transactivation of nuclear receptors, transcription of housekeeping genes
CSB and PCAF (zeige KAT2B Antikörper) 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 (zeige NR2C2 Antikörper)) 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 (zeige OGG1 Antikörper), 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