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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 19 Aug 2016.

Excision repair cross-complementing rodent repair deficiency, complementation group 6

CSB, Rad26, ERCC6
This gene encodes a DNA-binding protein that is important in transcription-coupled excision repair. The protein has ATP-stimulated ATPase activity; there are contradictory publications reporting presence or absence of helicase activity. The protein appears to interact with several transcription and excision repair proteins, and may promote complex formation at repair sites. Mutations in this gene result in Cockayne syndrome type B. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CsA, POLYMERASE, CAN, HAD, XPC
Papers using CSB antibodies
DNA damage stabilizes interaction of CSB with the transcription elongation machinery
Supplier
Vermeulen Wim et al., In The Journal of Cell Biology, 1999
... GFP-CSB was stably expressed in CS-B–deficient human fibroblasts (CS1AN-Sv) using SuperFect transfection reagent (QIAGEN).
Papers on CSB
Cockayne Syndrome-derived neurons display reduced synapse density and altered neural network synchrony.
New
Muotri et al., São Paulo, Brazil. In Hum Mol Genet, Feb 2016
The encoded ERCC6 protein is more commonly referred to as Cockayne Syndrome B protein (CSB).
The complete mitochondrial genome sequence of the tubeworm Lamellibrachia satsuma and structural conservation in the mitochondrial genome control regions of Order Sabellida.
New
Kim et al., Ansan, South Korea. In Mar Genomics, Feb 2016
We found 7 conserved sequence blocks (CSB), scattered throughout the control region of L. satsuma and other taxa of Annelida.
Novel missense mutations in a conserved loop between ERCC6 (CSB) helicase motifs V and VI: Insights into Cockayne syndrome.
New
Sutton et al., Newcastle upon Tyne, United Kingdom. In Am J Med Genet A, Feb 2016
UNASSIGNED: Cockayne syndrome is caused by biallelic ERCC8 (CSA) or ERCC6 (CSB) mutations and is characterized by growth restriction, microcephaly, developmental delay, and premature pathological aging.
Complete mitochondrial genome of black-banded trevally (Seriolina nigrofasciata): Genome characterization and phylogenetic considerations.
New
Wu et al., Changchun, China. In Mitochondrial Dna, Jan 2016
Two of the central conserved sequence blocks (CSB-2 and CSB-3) were identified and the core sequence (TACATGTATGTA) of terminal-associated sequences was recognized in the control region.
COCKAYNE SYNDROME: ROLE OF GENETIC COUNSELLING.
New
Sapkal et al., In J Ayub Med Coll Abbottabad, Jul 2015
Cockayne's Syndrome (CS) is a rare autosomal recessive disorder characterized by deficiency in the transcription-couple DNA repair pathway caused by mutations in the genes ERCC6 in 65% of individuals and ERCC8 in 35% of individuals.
Protein degradation pathways regulate the functions of helicases in the DNA damage response and maintenance of genomic stability.
Review
Brosh et al., Baltimore, United States. In Biomolecules, 2014
Turning to transcription, stability of the Cockayne Syndrome Group B DNA translocase (CSB) implicated in transcription-coupled repair (TCR) is regulated by a CSA ubiquitin ligase complex enabling recovery of RNA synthesis.
A high-fat diet and NAD(+) activate Sirt1 to rescue premature aging in cockayne syndrome.
Impact
Bohr et al., Baltimore, United States. In Cell Metab, 2014
Cockayne syndrome (CS) is an accelerated aging disorder characterized by progressive neurodegeneration caused by mutations in genes encoding the DNA repair proteins CS group A or B (CSA or CSB).
Regulation of active genome integrity and expression by Rad26p.
Review
Bhaumik et al., Carbondale, United States. In Nucleus, 2013
Both Rad26p and its human homolog CSB (Cockayne syndrome group B) are involved in regulation of chromatin structure, transcription and DNA repair.
Multiple interaction partners for Cockayne syndrome proteins: implications for genome and transcriptome maintenance.
Review
Stevnsner et al., Århus, Denmark. In Mech Ageing Dev, 2013
The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein.
The role of Cockayne syndrome group A (CSA) protein in transcription-coupled nucleotide excision repair.
Review
Saijo, Suita, Japan. In Mech Ageing Dev, 2013
Mutations in Cockayne syndrome groups A and B genes (CSA and CSB) result in defective TC-NER.
From laboratory tests to functional characterisation of Cockayne syndrome.
Review
Stefanini et al., Pavia, Italy. In Mech Ageing Dev, 2013
Furthermore, we provide a general overview of recent in vivo and in vitro studies indicating that both CSA and CSB proteins are involved in distinct aspects of the cellular responses to UV and oxidative stress, transcription and regulation of gene expression, chromatin remodelling, redox balance and cellular bioenergetics.
The conserved Cockayne syndrome B-piggyBac fusion protein (CSB-PGBD3) affects DNA repair and induces both interferon-like and innate antiviral responses in CSB-null cells.
GeneRIF
Weiner et al., Seattle, United States. In Dna Repair (amst), 2012
CSB-PGBD3 fusion protein is important in both health and disease, and could play a role in Cockayne syndrome.
A community-based study of nucleotide excision repair polymorphisms in relation to the risk of non-melanoma skin cancer.
GeneRIF
Alberg et al., Charleston, United States. In J Invest Dermatol, 2012
These hypothesis-generating findings suggest that functional variants in ERCC6 may be associated with an increased risk of non-melanoma skin cancer that may be specific to basal cell carcinoma.
UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair.
Impact
Marteijn et al., Rotterdam, Netherlands. In Nat Genet, 2012
The UVSSA protein interacts with elongating RNA polymerase II, localizes specifically to UV-induced lesions, resides in chromatin-associated TC-NER complexes and is implicated in stabilizing the TC-NER master organizing protein ERCC6 (also known as CSB) by delivering the deubiquitinating enzyme USP7 to TC-NER complexes.
Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair.
Impact
Tanaka et al., Ōsaka, Japan. In Nat Genet, 2012
Cockayne syndrome is a related disorder with defective TCR and consists of two complementation groups, Cockayne syndrome (CS)-A and CS-B, which are caused by mutations in ERCC8 (CSA) and ERCC6 (CSB), respectively.
Mutations in UVSSA cause UV-sensitive syndrome and impair RNA polymerase IIo processing in transcription-coupled nucleotide-excision repair.
Impact
Ogi et al., Nagasaki, Japan. In Nat Genet, 2012
Three of the seven known UV(S)S cases carry mutations in the Cockayne syndrome genes ERCC8 or ERCC6 (also known as CSA and CSB, respectively).
Cockayne syndrome group B protein prevents the accumulation of damaged mitochondria by promoting mitochondrial autophagy.
GeneRIF
Bohr et al., Baltimore, United States. In J Exp Med, 2012
report an increase in metabolism in the CSB(m/m) mouse model and CSB-deficient cells
Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome.
GeneRIF
Cleaver et al., San Francisco, United States. In Proc Natl Acad Sci U S A, 2012
Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a cs-b xp-c murine model of Cockayne syndrome
CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination.
GeneRIF
Proietti-De-Santis et al., Viterbo, Italy. In Cell Cycle, 2011
CSA and CSB are identified as the key elements of a regulatory mechanism that equilibrate beneficial and detrimental effects of p53 activity upon cellular stress.
Requirement of yeast RAD2, a homolog of human XPG gene, for efficient RNA polymerase II transcription. implications for Cockayne syndrome.
Impact
Prakash et al., Galveston, United States. In Cell, 2002
Inactivation of RAD26, the S. cerevisiae counterpart of the human CSB gene, also causes a deficiency in transcription, and a synergistic decline in transcription occurs in the absence of both the RAD2 and RAD26 genes.
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