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A Chromatin-Focused siRNA Screen for Regulators of p53-Dependent Transcription.

Sammons MA, Zhu J, Berger SL.

G3 (Bethesda). 2016 Aug 9;6(8):2671-8. doi: 10.1534/g3.116.031534.


DNA damage in the oligodendrocyte lineage and its role in brain aging.

Tse KH, Herrup K.

Mech Ageing Dev. 2016 May 26. pii: S0047-6374(16)30074-4. doi: 10.1016/j.mad.2016.05.006. [Epub ahead of print]


DNA excision repair at telomeres.

Jia P, Her C, Chai W.

DNA Repair (Amst). 2015 Dec;36:137-45. doi: 10.1016/j.dnarep.2015.09.017. Review.


Chromatin deregulation in disease.

Mirabella AC, Foster BM, Bartke T.

Chromosoma. 2016 Mar;125(1):75-93. doi: 10.1007/s00412-015-0530-0.


Reversal of mitochondrial defects with CSB-dependent serine protease inhibitors in patient cells of the progeroid Cockayne syndrome.

Chatre L, Biard DS, Sarasin A, Ricchetti M.

Proc Natl Acad Sci U S A. 2015 Jun 2;112(22):E2910-9. doi: 10.1073/pnas.1422264112.


Cockayne syndrome group B (Csb) and group a (Csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice.

Nagtegaal AP, Rainey RN, van der Pluijm I, Brandt RM, van der Horst GT, Borst JG, Segil N.

J Neurosci. 2015 Mar 11;35(10):4280-6. doi: 10.1523/JNEUROSCI.5063-14.2015.


Identification of two missense mutations of ERCC6 in three Chinese sisters with Cockayne syndrome by whole exome sequencing.

Yu S, Chen L, Ye L, Fei L, Tang W, Tian Y, Geng Q, Yi X, Xie J.

PLoS One. 2014 Dec 2;9(12):e113914. doi: 10.1371/journal.pone.0113914.


A novel ERCC6 splicing variant associated with a mild Cockayne syndrome phenotype.

Swartz JM, Akinci A, Andrew SF, Si─čirci A, Hirschhorn JN, Rosenfeld RG, Dauber A, Hwa V.

Horm Res Paediatr. 2014;82(5):344-52. doi: 10.1159/000368192.


A new mutation in the CSB gene in a Chinese patient with mild Cockayne syndrome.

Luo Y, Ling Y, Chen J, Xu X, Chen C, Leng F, Cheng J, Chen M, Lu Z.

Clin Case Rep. 2014 Apr;2(2):33-6. doi: 10.1002/ccr3.47.


ERCC6 dysfunction presenting as progressive neurological decline with brain hypomyelination.

Shehata L, Simeonov DR, Raams A, Wolfe L, Vanderver A, Li X, Huang Y, Garner S, Boerkoel CF, Thurm A, Herman GE, Tifft CJ, He M, Jaspers NG, Gahl WA.

Am J Med Genet A. 2014 Nov;164A(11):2892-900. doi: 10.1002/ajmg.a.36709.


The sequence-specific transcription factor c-Jun targets Cockayne syndrome protein B to regulate transcription and chromatin structure.

Lake RJ, Boetefuer EL, Tsai PF, Jeong J, Choi I, Won KJ, Fan HY.

PLoS Genet. 2014 Apr 17;10(4):e1004284. doi: 10.1371/journal.pgen.1004284.


ERCC6L2 mutations link a distinct bone-marrow-failure syndrome to DNA repair and mitochondrial function.

Tummala H, Kirwan M, Walne AJ, Hossain U, Jackson N, Pondarre C, Plagnol V, Vulliamy T, Dokal I.

Am J Hum Genet. 2014 Feb 6;94(2):246-56. doi: 10.1016/j.ajhg.2014.01.007.


Regulatory interplay of Cockayne syndrome B ATPase and stress-response gene ATF3 following genotoxic stress.

Kristensen U, Epanchintsev A, Rauschendorf MA, Laugel V, Stevnsner T, Bohr VA, Coin F, Egly JM.

Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):E2261-70. doi: 10.1073/pnas.1220071110.


Structure, function and regulation of CSB: a multi-talented gymnast.

Lake RJ, Fan HY.

Mech Ageing Dev. 2013 May-Jun;134(5-6):202-11. doi: 10.1016/j.mad.2013.02.004. Review.


A possible cranio-oro-facial phenotype in Cockayne syndrome.

Bloch-Zupan A, Rousseaux M, Laugel V, Schmittbuhl M, Mathis R, Desforges E, Koob M, Zaloszyc A, Dollfus H, Laugel V.

Orphanet J Rare Dis. 2013 Jan 14;8:9. doi: 10.1186/1750-1172-8-9.


Cockayne Syndrome group B protein interacts with TRF2 and regulates telomere length and stability.

Batenburg NL, Mitchell TR, Leach DM, Rainbow AJ, Zhu XD.

Nucleic Acids Res. 2012 Oct;40(19):9661-74. doi: 10.1093/nar/gks745.


Human Cockayne syndrome B protein reciprocally communicates with mitochondrial proteins and promotes transcriptional elongation.

Berquist BR, Canugovi C, Sykora P, Wilson DM 3rd, Bohr VA.

Nucleic Acids Res. 2012 Sep 1;40(17):8392-405.


Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome.

Revet I, Feeney L, Tang AA, Huang EJ, Cleaver JE.

Proc Natl Acad Sci U S A. 2012 Mar 20;109(12):4627-32. doi: 10.1073/pnas.1202621109.


Testicular nuclear receptor 4 (TR4) regulates UV light-induced responses via Cockayne syndrome B protein-mediated transcription-coupled DNA repair.

Liu S, Yan SJ, Lee YF, Liu NC, Ting HJ, Li G, Wu Q, Chen LM, Chang C.

J Biol Chem. 2011 Nov 4;286(44):38103-8. doi: 10.1074/jbc.M111.259523.

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