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Articles, Neurobiology of Disease

Ubiquitin-Activating Enzyme Activity Contributes to Differential Accumulation of Mutant Huntingtin in Brain and Peripheral Tissues

Brandy E. Wade, Chuan-En Wang, Sen Yan, Kavita Bhat, Brenda Huang, Shihua Li and Xiao-Jiang Li
Journal of Neuroscience 18 June 2014, 34 (25) 8411-8422; https://doi.org/10.1523/JNEUROSCI.0775-14.2014
Brandy E. Wade
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
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Chuan-En Wang
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
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Sen Yan
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
2State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Chaoyang District, Beijing 100101, People's Republic of China
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Kavita Bhat
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
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Brenda Huang
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
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Shihua Li
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
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Xiao-Jiang Li
1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
2State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Chaoyang District, Beijing 100101, People's Republic of China
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Article Information

DOI 
https://doi.org/10.1523/JNEUROSCI.0775-14.2014
PubMed 
24948797
Published By 
Society for Neuroscience
History 
  • Received February 25, 2014
  • Revision received April 24, 2014
  • Accepted April 29, 2014
  • First published June 18, 2014.
  • Version of record published June 18, 2014.
Copyright & Usage 
Copyright © 2014 the authors 0270-6474/14/348411-12$15.00/0

Author Information

  1. Brandy E. Wade1,
  2. Chuan-En Wang1,
  3. Sen Yan1,2,
  4. Kavita Bhat1,
  5. Brenda Huang1,
  6. Shihua Li1, and
  7. Xiao-Jiang Li1,2
  1. 1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, and
  2. 2State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Chaoyang District, Beijing 100101, People's Republic of China
View Full Text

Author contributions

  1. Author contributions: B.E.W., S.L., and X.-J.L. designed research; B.E.W., C.-E.W., S.Y., K.B., and B.H. performed research; B.E.W. and X.-J.L. analyzed data; B.E.W., S.L., and X.-J.L. wrote the paper.

Disclosures

    • Received February 25, 2014.
    • Revision received April 24, 2014.
    • Accepted April 29, 2014.
  • This work was supported by National Institutes of Health Grants AG19206 and NS041449 (to X.-J.L.); and AG031153 and NS0405016 (to S.L.). This research project was supported in part by the Viral Vector Core of the Emory National Institute of Neurological Disorders and Stroke Core Facilities Grant P30NS055077. We thank Matthew Randolph for technical assistance in muscle injection and stereotaxic surgery, Dr. Michael Epstein for recommendations in statistical analysis, Cheryl Strauss for critically reading this manuscript, and Dr. Nick Seyfried for the SUMO-2/3 antibody.

  • The authors declare no competing financial interests.

  • Correspondence should be addressed to Dr. Xiao-Jiang Li, Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Room 347, Atlanta, GA 30322. xli2{at}emory.edu

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The Journal of Neuroscience: 34 (25)
Journal of Neuroscience
Vol. 34, Issue 25
18 Jun 2014
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Ubiquitin-Activating Enzyme Activity Contributes to Differential Accumulation of Mutant Huntingtin in Brain and Peripheral Tissues
Brandy E. Wade, Chuan-En Wang, Sen Yan, Kavita Bhat, Brenda Huang, Shihua Li, Xiao-Jiang Li
Journal of Neuroscience 18 June 2014, 34 (25) 8411-8422; DOI: 10.1523/JNEUROSCI.0775-14.2014

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Ubiquitin-Activating Enzyme Activity Contributes to Differential Accumulation of Mutant Huntingtin in Brain and Peripheral Tissues
Brandy E. Wade, Chuan-En Wang, Sen Yan, Kavita Bhat, Brenda Huang, Shihua Li, Xiao-Jiang Li
Journal of Neuroscience 18 June 2014, 34 (25) 8411-8422; DOI: 10.1523/JNEUROSCI.0775-14.2014
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Keywords

  • misfolding
  • neurodegneration
  • ubiquitin

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