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Select item 77449551.

Cockayne syndrome proteins CSA and CSB maintain mitochondrial homeostasis through NAD⁺ signaling

Mustafa N. Okur, Evandro F. Fang, Elayne M. Fivenson, Vinod Tiwari, Deborah L. Croteau, Vilhelm A. Bohr

Aging Cell. 2020 Dec; 19(12): e13268. Published online 2020 Nov 9. doi: 10.1111/acel.13268

PMCID:: PMC7744955

Abstract Article PDF–1.8M

Select item 72270282.

Mouse Models of Inherited Retinal Degeneration with Photoreceptor Cell Loss

Gayle B. Collin, Navdeep Gogna, Bo Chang, Nattaya Damkham, Jai Pinkney, Lillian F. Hyde, Lisa Stone, Jürgen K. Naggert, Patsy M. Nishina, Mark P. Krebs

Cells. 2020 Apr; 9(4): 931. Published online 2020 Apr 10. doi: 10.3390/cells9040931

PMCID:: PMC7227028

Abstract Article PDF–5.4M

Select item 71449633.

XPF-ERCC1 protects liver, kidney and blood homeostasis outside the canonical excision repair pathways

Lee Mulderrig, Juan I. Garaycoechea

PLoS Genet. 2020 Apr; 16(4): e1008555. Published online 2020 Apr 9. doi: 10.1371/journal.pgen.1008555

PMCID:: PMC7144963

Abstract Article PDF–3.1M

Select item 66756124.

DNA crosslink repair safeguards genomic stability during pre-meiotic germ cell development

Ross J Hill, Gerry P Crossan

Nat Genet. Author manuscript; available in PMC 2020 Jan 31.

Published in final edited form as: Nat Genet. 2019 Jul 4; 51(8): 1283–1294. Published online 2019 Jul 31. doi: 10.1038/s41588-019-0471-2

PMCID:: PMC6675612

Abstract Article PDF–3.5M

Select item 48466725.

Mutations in Cockayne Syndrome-Associated Genes (Csa and Csb) Predispose to Cisplatin-Induced Hearing Loss in Mice

Robert N. Rainey, Sum-yan Ng, Juan Llamas, Gijsbertus T. J. van der Horst, Neil Segil

J Neurosci. 2016 Apr 27; 36(17): 4758–4770. doi: 10.1523/JNEUROSCI.3890-15.2016

PMCID:: PMC4846672

Abstract Article PDF–5.2M

Select item 46258376.

Defective Mitophagy in XPA via PARP1 Hyperactivation and NAD+/SIRT1 Reduction

Evandro Fei Fang, Morten Scheibye-Knudsen, Lear E. Brace, Henok Kassahun, Tanima SenGupta, Hilde Nilsen, James R. Mitchell, Deborah L. Croteau, Vilhelm A. Bohr

Cell. Author manuscript; available in PMC 2015 Oct 29.

Published in final edited form as: Cell. 2014 May 8; 157(4): 882–896. doi: 10.1016/j.cell.2014.03.026

PMCID:: PMC4625837

Abstract Article PDF–3.0M

Select item 44860897.

Nuclear myosin 1 contributes to a chromatin landscape compatible with RNA polymerase II transcription activation

Bader Almuzzaini, Aishe A. Sarshad, Ann-Kristin Östlund Farrants, Piergiorgio Percipalle

BMC Biol. 2015; 13: 35. Published online 2015 Jun 5. doi: 10.1186/s12915-015-0147-z

PMCID:: PMC4486089

Abstract Article PDF–2.6M

Select item 43823898.

The transcription coupled repair protein ERCC6/CSB also protects against repeat expansion in a mouse model of the Fragile X premutation

Xiao-Nan Zhao, Karen Usdin

Hum Mutat. Author manuscript; available in PMC 2016 Apr 1.

Published in final edited form as: Hum Mutat. 2015 Apr; 36(4): 482–487. doi: 10.1002/humu.22777

PMCID:: PMC4382389

Abstract Article PDF–536K

Select item 43551999.

Cockayne Syndrome Group B (Csb) and Group A (Csa) Deficiencies Predispose to Hearing Loss and Cochlear Hair Cell Degeneration in Mice

A. Paul Nagtegaal, Robert N. Rainey, Ingrid van der Pluijm, Renata M.C. Brandt, Gijsbertus T.J. van der Horst, J. Gerard G. Borst, Neil Segil

J Neurosci. 2015 Mar 11; 35(10): 4280–4286. doi: 10.1523/JNEUROSCI.5063-14.2015

PMCID:: PMC4355199

Abstract Article PDF–4.7M

Select item 426173510.

A High Fat Diet and NAD⁺ Rescue Premature Aging in Cockayne Syndrome

Morten Scheibye-Knudsen, Sarah J. Mitchell, Evandro F. Fang, Teruaki Iyama, Theresa Ward, James Wang, Christopher A. Dunn, Nagendra Singh, Sebastian Veith, M. Mahdi Hasan, Aswin Mangerich, Mark A. Wilson, Mark P. Mattson, Linda H. Bergersen, Victoria C. Cogger, Alessandra Warren, David G. Le Couteur, Ruin Moaddel, David M. Wilson, 3rd, Deborah L. Croteau, Rafael de Cabo, Vilhelm A. Bohr

Cell Metab. Author manuscript; available in PMC 2015 Nov 4.

Published in final edited form as: Cell Metab. 2014 Nov 4; 20(5): 840–855. Published online 2014 Nov 4. doi: 10.1016/j.cmet.2014.10.005

PMCID:: PMC4261735

Abstract Article PDF–3.5M

Select item 421003711.

Dysregulation of gene expression as a cause of Cockayne syndrome neurological disease

Yuming Wang, Probir Chakravarty, Michael Ranes, Gavin Kelly, Philip J. Brooks, Edward Neilan, Aengus Stewart, Giampietro Schiavo, Jesper Q. Svejstrup

Proc Natl Acad Sci U S A. 2014 Oct 7; 111(40): 14454–14459. Published online 2014 Sep 23. doi: 10.1073/pnas.1412569111

PMCID:: PMC4210037

Abstract Article PDF–1.5M

Select item 406746612.

Gender and cell-type specific effects of the transcription coupled repair protein, ERCC6/CSB, on repeat expansion in a mouse model of the Fragile X-related disorders

Xiao-Nan Zhao, Karen Usdin

Hum Mutat. Author manuscript; available in PMC 2015 Mar 1.

Published in final edited form as: Hum Mutat. 2014 Mar; 35(3): 341–349. doi: 10.1002/humu.22495

PMCID:: PMC4067466

Abstract Article PDF–1.1M

Select item 364688213.

The Chromatin Remodeling Factor CSB Recruits Histone Acetyltransferase PCAF to rRNA Gene Promoters in Active State for Transcription Initiation

Meili Shen, Tingting Zhou, Wenbing Xie, Te Ling, Qiaoyun Zhu, Le Zong, Guoliang Lyu, Qianqian Gao, Feixiong Zhang, Wei Tao

PLoS One. 2013; 8(5): e62668. Published online 2013 May 7. doi: 10.1371/journal.pone.0062668

PMCID:: PMC3646882

Abstract Article PDF–2.5M

Select item 360510314.

Nuclear Myosin 1c Facilitates the Chromatin Modifications Required to Activate rRNA Gene Transcription and Cell Cycle Progression

Aishe Sarshad, Fatemeh Sadeghifar, Emilie Louvet, Raffaele Mori, Stefanie Böhm, Bader Al-Muzzaini, Anna Vintermist, Nathalie Fomproix, Ann-Kristin Östlund, Piergiorgio Percipalle

PLoS Genet. 2013 Mar; 9(3): e1003397. Published online 2013 Mar 21. doi: 10.1371/journal.pgen.1003397

PMCID:: PMC3605103

Abstract Article PDF–3.5M

Select item 357241015.

A conditional knockout resource for the genome–wide study of mouse gene function

William C. Skarnes, Barry Rosen, Anthony P. West, Manousos Koutsourakis, Wendy Bushell, Vivek Iyer, Alejandro O. Mujica, Mark Thomas, Jennifer Harrow, Tony Cox, David Jackson, Jessica Severin, Patrick Biggs, Jun Fu, Michael Nefedov, Pieter J. de Jong, A. Francis Stewart, Allan Bradley

Nature. Author manuscript; available in PMC 2013 Feb 14.

Published in final edited form as: Nature. 2011 Jun 15; 474(7351): 337–342. Published online 2011 Jun 15. doi: 10.1038/nature10163

PMCID:: PMC3572410

Abstract Article PDF–1.1M

Select item 332835916.

Cockayne syndrome group B protein prevents the accumulation of damaged mitochondria by promoting mitochondrial autophagy

Morten Scheibye-Knudsen, Mahesh Ramamoorthy, Peter Sykora, Scott Maynard, Ping-Chang Lin, Robin K. Minor, David M. Wilson III, Marcus Cooper, Richard Spencer, Rafael de Cabo, Deborah L. Croteau, Vilhelm A. Bohr

J Exp Med. 2012 Apr 9; 209(4): 855–869. doi: 10.1084/jem.20111721

PMCID:: PMC3328359

Abstract Article PDF–3.9M

Select item 331138717.

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

Ingrid Revet, Luzviminda Feeney, Amy A. Tang, Eric J. Huang, James E. Cleaver

Proc Natl Acad Sci U S A. 2012 Mar 20; 109(12): 4627–4632. Published online 2012 Mar 5. doi: 10.1073/pnas.1202621109

PMCID:: PMC3311387

Abstract Article PDF–671K

Select item 328699418.

Defective transcription initiation causes postnatal growth failure in a mouse model of nucleotide excision repair (NER) progeria

Irene Kamileri, Ismene Karakasilioti, Aria Sideri, Theodoros Kosteas, Antonis Tatarakis, Iannis Talianidis, George A. Garinis

Proc Natl Acad Sci U S A. 2012 Feb 21; 109(8): 2995–3000. Published online 2012 Feb 8. doi: 10.1073/pnas.1114941109

PMCID:: PMC3286994

Abstract Article PDF–725K

Select item 323422019.

Dick Jaarsma, Ingrid van der Pluijm, Monique C. de Waard, Elize D. Haasdijk, Renata Brandt, Marcel Vermeij, Yvonne Rijksen, Alex Maas, Harry van Steeg, Jan H. J. Hoeijmakers, Gijsbertus T. J. van der Horst

PLoS Genet. 2011 Dec; 7(12): e1002405. Published online 2011 Dec 8. doi: 10.1371/journal.pgen.1002405

PMCID:: PMC3234220

Abstract Article PDF–1.2M

Select item 320746420.

Testicular Nuclear Receptor 4 (TR4) Regulates UV Light-induced Responses via Cockayne Syndrome B Protein-mediated Transcription-coupled DNA Repair

Su Liu, Shian-Jang Yan, Yi-Fen Lee, Ning-Chun Liu, Huei-Ju Ting, Gonghui Li, Qiao Wu, Lu-Min Chen, Chawnshang Chang

J Biol Chem. 2011 Nov 4; 286(44): 38103–38108. Published online 2011 Sep 14. doi: 10.1074/jbc.M111.259523

PMCID:: PMC3207464

Abstract Article PDF–688K

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