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Articles, Neurobiology of Disease

Loss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein Clearance

Angela M. Jablonski, Todd Lamitina, Nicole F. Liachko, Mariangela Sabatella, Jiayin Lu, Lei Zhang, Lyle W. Ostrow, Preetika Gupta, Chia-Yen Wu, Shachee Doshi, Jelena Mojsilovic-Petrovic, Hannes Lans, Jiou Wang, Brian Kraemer and Robert G. Kalb
Journal of Neuroscience 21 October 2015, 35 (42) 14286-14306; https://doi.org/10.1523/JNEUROSCI.0642-15.2015
Angela M. Jablonski
1Department of Neuroscience, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
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Todd Lamitina
4Department of Pediatrics and Cell Biology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15213,
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Nicole F. Liachko
5Geriatric Research Education and Clinical Center, Veterans Affairs Puget Sound Health Care System, Department of Medicine, University of Washington, Seattle, Washington 98195,
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Mariangela Sabatella
6Department of Genetics, Erasmus Medical Centre, Cancer Genomics Institute, Rotterdam 3015 CN, The Netherlands,
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  • ORCID record for Mariangela Sabatella
Jiayin Lu
7Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, and
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Lei Zhang
2Department of Pediatrics, Division of Neurology, Research Institute, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104,
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Lyle W. Ostrow
8Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
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Preetika Gupta
1Department of Neuroscience, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
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Chia-Yen Wu
2Department of Pediatrics, Division of Neurology, Research Institute, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104,
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Shachee Doshi
1Department of Neuroscience, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
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Jelena Mojsilovic-Petrovic
2Department of Pediatrics, Division of Neurology, Research Institute, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104,
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Hannes Lans
6Department of Genetics, Erasmus Medical Centre, Cancer Genomics Institute, Rotterdam 3015 CN, The Netherlands,
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  • ORCID record for Hannes Lans
Jiou Wang
7Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, and
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Brian Kraemer
6Department of Genetics, Erasmus Medical Centre, Cancer Genomics Institute, Rotterdam 3015 CN, The Netherlands,
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Robert G. Kalb
2Department of Pediatrics, Division of Neurology, Research Institute, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104,
3Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
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Article Information

DOI 
https://doi.org/10.1523/JNEUROSCI.0642-15.2015
PubMed 
26490867
Published By 
Society for Neuroscience
History 
  • Received February 16, 2015
  • Revision received August 31, 2015
  • Accepted September 8, 2015
  • First published October 21, 2015.
  • Version of record published October 21, 2015.
Copyright & Usage 
Copyright © 2015 the authors 0270-6474/15/3514286-21$15.00/0

Author Information

  1. Angela M. Jablonski1,
  2. Todd Lamitina4,
  3. Nicole F. Liachko5,
  4. Mariangela Sabatella6,
  5. Jiayin Lu7,
  6. Lei Zhang2,
  7. Lyle W. Ostrow8,
  8. Preetika Gupta1,
  9. Chia-Yen Wu2,
  10. Shachee Doshi1,
  11. Jelena Mojsilovic-Petrovic2,
  12. Hannes Lans6,
  13. Jiou Wang7,
  14. Brian Kraemer6, and
  15. Robert G. Kalb2,3
  1. 1Department of Neuroscience, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
  2. 2Department of Pediatrics, Division of Neurology, Research Institute, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104,
  3. 3Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104,
  4. 4Department of Pediatrics and Cell Biology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania 15213,
  5. 5Geriatric Research Education and Clinical Center, Veterans Affairs Puget Sound Health Care System, Department of Medicine, University of Washington, Seattle, Washington 98195,
  6. 6Department of Genetics, Erasmus Medical Centre, Cancer Genomics Institute, Rotterdam 3015 CN, The Netherlands,
  7. 7Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, and
  8. 8Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
View Full Text

Author contributions

  1. Author contributions: A.M.J., T.L., N.F.L., M.S., J.L., L.Z., L.W.O., C.-Y.W., H.L., J.W., B.K., and R.G.K. designed research; A.M.J., T.L., N.F.L., M.S., J.L., L.Z., P.G., C.-Y.W., S.D., H.L., J.W., and B.K. performed research; A.M.J., L.W.O., and J.M.-P. contributed unpublished reagents/analytic tools; A.M.J., T.L., N.F.L., M.S., J.L., P.G., C.-Y.W., S.D., H.L., J.W., B.K., and R.G.K. analyzed data; A.M.J. and R.G.K. wrote the paper.

Disclosures

    • Received February 16, 2015.
    • Revision received August 31, 2015.
    • Accepted September 8, 2015.
  • This work was supported by NIH Grants R01-NS052325 and R21-NS077909 to R.G.K., F31-NS07726 to A.M.J., and FP7 Marie Curie International Training Network Address 316390 to M.S. The RAD-23 fosmid was obtained from the C. elegans TransgeneOme project. Some strains were provided by the Caenorhabditis Genetics Center, which is funded by National Institutes of Health Office of Research Infrastructure Programs P40-OD010440, and the National Bioresource Project for the Nematode. We thank Ulrich Hartl for the CPY*-GFP plasmid; Steven Grossman for the RAD-23 plasmids; Johns Ravits and Jeff Rothstein for providing human tissue; Maria Lim, Elizabeth Morton, Zissimos Mourelatos, and Predrag Krajacic for assistance; the Target ALS Human Postmortem Tissue Core for providing postmortem tissue samples; and Harry Ischiropolous, Kelly Jordan-Sciutto, Alice Chen-Plotkin, and Raymond Roos for guidance and helpful discussion.

  • The authors declare no competing financial interests.

  • Correspondence should be addressed to Dr. Robert G. Kalb, Research Institute, Children's Hospital of Philadelphia, 3615 Civic Center Blvd, Philadelphia, PA 19104. kalb{at}email.chop.edu

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Feb 2025154011
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Apr 202552010
May 2025112615
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Jul 202582310
Aug 2025103112
Sep 2025204635
Oct 2025142910
Nov 2025191
Total 2025112309144
Total118138521550
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The Journal of Neuroscience: 35 (42)
Journal of Neuroscience
Vol. 35, Issue 42
21 Oct 2015
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Loss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein Clearance
Angela M. Jablonski, Todd Lamitina, Nicole F. Liachko, Mariangela Sabatella, Jiayin Lu, Lei Zhang, Lyle W. Ostrow, Preetika Gupta, Chia-Yen Wu, Shachee Doshi, Jelena Mojsilovic-Petrovic, Hannes Lans, Jiou Wang, Brian Kraemer, Robert G. Kalb
Journal of Neuroscience 21 October 2015, 35 (42) 14286-14306; DOI: 10.1523/JNEUROSCI.0642-15.2015

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Loss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein Clearance
Angela M. Jablonski, Todd Lamitina, Nicole F. Liachko, Mariangela Sabatella, Jiayin Lu, Lei Zhang, Lyle W. Ostrow, Preetika Gupta, Chia-Yen Wu, Shachee Doshi, Jelena Mojsilovic-Petrovic, Hannes Lans, Jiou Wang, Brian Kraemer, Robert G. Kalb
Journal of Neuroscience 21 October 2015, 35 (42) 14286-14306; DOI: 10.1523/JNEUROSCI.0642-15.2015
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Keywords

  • aging
  • ALS
  • motor neuron disease
  • neurodegeneration
  • proteotoxicity
  • RAD-23

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