Property Summary

NCBI Gene PubMed Count 165
Grant Count 75
R01 Count 26
Funding $47,890,449.9
PubMed Score 269.29
PubTator Score 188.26

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (3)

Disease log2 FC p
malignant mesothelioma 1.200 0.000
osteosarcoma -1.191 0.000
tuberculosis -1.300 0.000

Synonym

Accession Q03468 D3DX94 Q5W0L9
Symbols CSB
CKN2
COFS
ARMD5
COFS1
POF11
RAD26
UVSS1

Gene

PDB

4CVO  

Gene RIF (134)

PMID Text
26826127 new role of VCP/p97 segregase in the timely processing of ubiquitinated CSB from damaged chromatin.
26649138 The frequency of the XPD 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.
26620705 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.
26616585 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.
26578602 CSB and CTCF can regulate each other's chromatin association, thereby modulating chromatin structure and coordinating gene expression in response to oxidative stress.
26100862 Data indicate that Cockayne syndrome group B protein CSB function is necessary for the recruitment of recombinational factors.
26030138 33 proteins that were not previously known to interact with CSB.
25731027 The polymorphism of DNA repair genes XPD, XRCC1, OGG1, and ERCC6, life expectancy, and the inclination to smoke
25484185 These studies have provided significant functional and mechanistic insights of Rad26p/CSB in regulation of gene expression and genome integrity as described here.
25463447 The present report describes a case of Cockayne syndrome in a Chinese family, with the patients carrying two missense mutations (c.1595A>G, p.Asp532Gly and c.1607T>G, p.Leu536Trp) in the ERCC6 gene in an apparently compound heterozygote.
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AA Sequence

MPNEGIPHSSQTQEQDCLQSQPVSNNEEMAIKQESGGDGEVEEYLSFRSVGDGLSTSAVGCASAAPRRGP      1 - 70
ALLHIDRHQIQAVEPSAQALELQGLGVDVYDQDVLEQGVLQQVDNAIHEASRASQLVDVEKEYRSVLDDL     71 - 140
TSCTTSLRQINKIIEQLSPQAATSRDINRKLDSVKRQKYNKEQQLKKITAKQKHLQAILGGAEVKIELDH    141 - 210
ASLEEDAEPGPSSLGSMLMPVQETAWEELIRTGQMTPFGTQIPQKQEKKPRKIMLNEASGFEKYLADQAK    211 - 280
LSFERKKQGCNKRAARKAPAPVTPPAPVQNKNKPNKKARVLSKKEERLKKHIKKLQKRALQFQGKVGLPK    281 - 350
ARRPWESDMRPEAEGDSEGEESEYFPTEEEEEEEDDEVEGAEADLSGDGTDYELKPLPKGGKRQKKVPVQ    351 - 420
EIDDDFFPSSGEEAEAASVGEGGGGGRKVGRYRDDGDEDYYKQRLRRWNKLRLQDKEKRLKLEDDSEESD    421 - 490
AEFDEGFKVPGFLFKKLFKYQQTGVRWLWELHCQQAGGILGDEMGLGKTIQIIAFLAGLSYSKIRTRGSN    491 - 560
YRFEGLGPTVIVCPTTVMHQWVKEFHTWWPPFRVAILHETGSYTHKKEKLIRDVAHCHGILITSYSYIRL    561 - 630
MQDDISRYDWHYVILDEGHKIRNPNAAVTLACKQFRTPHRIILSGSPMQNNLRELWSLFDFIFPGKLGTL    631 - 700
PVFMEQFSVPITMGGYSNASPVQVKTAYKCACVLRDTINPYLLRRMKSDVKMSLSLPDKNEQVLFCRLTD    701 - 770
EQHKVYQNFVDSKEVYRILNGEMQIFSGLIALRKICNHPDLFSGGPKNLKGLPDDELEEDQFGYWKRSGK    771 - 840
MIVVESLLKIWHKQGQRVLLFSQSRQMLDILEVFLRAQKYTYLKMDGTTTIASRQPLITRYNEDTSIFVF    841 - 910
LLTTRVGGLGVNLTGANRVVIYDPDWNPSTDTQARERAWRIGQKKQVTVYRLLTAGTIEEKIYHRQIFKQ    911 - 980
FLTNRVLKDPKQRRFFKSNDLYELFTLTSPDASQSTETSAIFAGTGSDVQTPKCHLKRRIQPAFGADHDV    981 - 1050
PKRKKFPASNISVNDATSSEEKSEAKGAEVNAVTSNRSDPLKDDPHMSSNVTSNDRLGEETNAVSGPEEL   1051 - 1120
SVISGNGECSNSSGTGKTSMPSGDESIDEKLGLSYKRERPSQAQTEAFWENKQMENNFYKHKSKTKHHSV   1121 - 1190
AEEETLEKHLRPKQKPKNSKHCRDAKFEGTRIPHLVKKRRYQKQDSENKSEAKEQSNDDYVLEKLFKKSV   1191 - 1260
GVHSVMKHDAIMDGASPDYVLVEAEANRVAQDALKALRLSRQRCLGAVSGVPTWTGHRGISGAPAGKKSR   1261 - 1330
FGKKRNSNFSVQHPSSTSPTEKCQDGIMKKEGKDNVPEHFSGRAEDADSSSGPLASSSLLAKMRARNHLI   1331 - 1400
LPERLESESGHLQEASALLPTTEHDDLLVEMRNFIAFQAHTDGQASTREILQEFESKLSASQSCVFRELL   1401 - 1470
RNLCTFHRTSGGEGIWKLKPEYC                                                  1471 - 1493
//

Text Mined References (175)

PMID Year Title
26826127 2016 Valosin-containing Protein (VCP)/p97 Segregase Mediates Proteolytic Processing of Cockayne Syndrome Group B (CSB) in Damaged Chromatin.
26649138 2016 The Cellular Response to Oxidatively Induced DNA Damage and Polymorphism of Some DNA Repair Genes Associated with Clinicopathological Features of Bladder Cancer.
26620705 2016 The C-terminal Region and SUMOylation of Cockayne Syndrome Group B Protein Play Critical Roles in Transcription-coupled Nucleotide Excision Repair.
26616585 2016 Elements That Regulate the DNA Damage Response of Proteins Defective in Cockayne Syndrome.
26578602 2016 The CSB chromatin remodeler and CTCF architectural protein cooperate in response to oxidative stress.
26218421 2015 CSB-PGBD3 Mutations Cause Premature Ovarian Failure.
26100862 2015 DNA damage during the G0/G1 phase triggers RNA-templated, Cockayne syndrome B-dependent homologous recombination.
26030138 2015 Identification of Novel Proteins Co-Purifying with Cockayne Syndrome Group B (CSB) Reveals Potential Roles for CSB in RNA Metabolism and Chromatin Dynamics.
25731027 2014 [The polymorphism of DNA repair genes XPD, XRCC1, OGG1, and ERCC6, life expectancy, and the inclination to smoke].
25484185 2014 Regulation of active genome integrity and expression by Rad26p.
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