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Brief Communications

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 and Neil Segil
Journal of Neuroscience 11 March 2015, 35 (10) 4280-4286; https://doi.org/10.1523/JNEUROSCI.5063-14.2015
A. Paul Nagtegaal
1Departments of Neuroscience and
2Otorhinolaryngology, and
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  • ORCID record for A. Paul Nagtegaal
Robert N. Rainey
4Departments of Regenerative Medicine/Stem Cell Biology, and Otolaryngology, Eli and Edythe Broad Center, University of Southern California, Los Angeles, California 90033
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Ingrid van der Pluijm
3Department of Genetics, Cancer Genomic Center, Erasmus MC, 3015 CN, Rotterdam, The Netherlands, and
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Renata M.C. Brandt
3Department of Genetics, Cancer Genomic Center, Erasmus MC, 3015 CN, Rotterdam, The Netherlands, and
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Gijsbertus T.J. van der Horst
3Department of Genetics, Cancer Genomic Center, Erasmus MC, 3015 CN, Rotterdam, The Netherlands, and
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J. Gerard G. Borst
1Departments of Neuroscience and
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Neil Segil
4Departments of Regenerative Medicine/Stem Cell Biology, and Otolaryngology, Eli and Edythe Broad Center, University of Southern California, Los Angeles, California 90033
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Article Information

DOI 
https://doi.org/10.1523/JNEUROSCI.5063-14.2015
PubMed 
25762674
Published By 
Society for Neuroscience
History 
  • Received December 13, 2014
  • Revision received February 4, 2015
  • Accepted February 5, 2015
  • First published March 11, 2015.
  • Version of record published March 11, 2015.
Copyright & Usage 
Copyright © 2015 the authors 0270-6474/15/354280-07$15.00/0

Author Information

  1. A. Paul Nagtegaal1,2,*,
  2. Robert N. Rainey4,*,
  3. Ingrid van der Pluijm3,
  4. Renata M.C. Brandt3,
  5. Gijsbertus T.J. van der Horst3,
  6. J. Gerard G. Borst1, and
  7. Neil Segil4
  1. 1Departments of Neuroscience and
  2. 2Otorhinolaryngology, and
  3. 3Department of Genetics, Cancer Genomic Center, Erasmus MC, 3015 CN, Rotterdam, The Netherlands, and
  4. 4Departments of Regenerative Medicine/Stem Cell Biology, and Otolaryngology, Eli and Edythe Broad Center, University of Southern California, Los Angeles, California 90033
  • I. van der Pluijm's present address: Department of Vascular Surgery, Erasmus MC, Dr. Molewaterplein 50, 3015 GE, Rotterdam, The Netherlands.

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Author contributions

  1. Author contributions: A.P.N., R.N.R., G.T.J.v.d.H., J.G.G.B., and N.S. designed research; A.P.N., R.N.R., I.v.d.P., and R.M.C.B. performed research; A.P.N., R.N.R., I.v.d.P., R.M.C.B., G.T.J.v.d.H., J.G.G.B., and N.S. analyzed data; A.P.N., R.N.R., G.T.J.v.d.H., J.G.G.B., and N.S. wrote the paper.

  2. ↵*A.P.N. and R.N.R. are co-first authors and contributed equally to this work.

  • I. van der Pluijm's present address: Department of Vascular Surgery, Erasmus MC, Dr. Molewaterplein 50, 3015 GE, Rotterdam, The Netherlands.

Disclosures

    • Received December 13, 2014.
    • Revision received February 4, 2015.
    • Accepted February 5, 2015.
  • This work was supported by National Institutes of Health (NIH)-Ruth L. Kirschstein National Research Service Award F32DC010125 (to R.N.R.), the Sidgmore Family Foundation, NIH Grant R01-DC-007173 (to N.S.), the Dutch Fund for Economic Structure Reinforcement (Grant 0908 for the “NeuroBasic PharmaPhenomics project,” to J.G.G.B.), and the Heinsius-Houbolt Fund (A.P.N.). We thank Juan Llamas, Welly Makmura, and Francesca Della Ripa for excellent technical support; and Caroline Abdala for assistance with the ABR and DPOAE analyses.

  • The authors declare no competing financial interests.

  • Correspondence should be addressed to either of the following: Neil Segil, 1425 San Pablo Street, BCC211, Los Angeles, CA 90033, nsegil{at}med.usc.edu; or Gerard G. Borst, Wytemaweg 80, 3015 CN, Rotterdam, The Netherlands, g.borst{at}erasmusmc.nl

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Total 202522285130
Total3091755833
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The Journal of Neuroscience: 35 (10)
Journal of Neuroscience
Vol. 35, Issue 10
11 Mar 2015
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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
Journal of Neuroscience 11 March 2015, 35 (10) 4280-4286; DOI: 10.1523/JNEUROSCI.5063-14.2015

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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
Journal of Neuroscience 11 March 2015, 35 (10) 4280-4286; DOI: 10.1523/JNEUROSCI.5063-14.2015
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Keywords

  • Cockayne syndrome
  • csb/csa
  • DNA damage
  • DNA repair
  • hair cell
  • hearing loss

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