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Research ArticleResearch Article: New Research, Disorders of the Nervous System

Gordon Holmes Syndrome Model Mice Exhibit Alterations in Microglia, Age, and Sex-Specific Disruptions in Cognitive and Proprioceptive Function

Arlene J. George, Wei Wei, Dhanya N. Pyaram, Morgan Gomez, Nitheyaa Shree, Jayashree Kadirvelu, Hannah Lail, Desiree Wanders, Anne Z. Murphy and Angela M. Mabb
eNeuro 27 December 2023, 11 (1) ENEURO.0074-23.2023; https://doi.org/10.1523/ENEURO.0074-23.2023
Arlene J. George
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
2Center for Behavioral Neuroscience, Georgia State University, Atlanta 30303, Georgia
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Wei Wei
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
2Center for Behavioral Neuroscience, Georgia State University, Atlanta 30303, Georgia
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Dhanya N. Pyaram
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
2Center for Behavioral Neuroscience, Georgia State University, Atlanta 30303, Georgia
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Morgan Gomez
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
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Nitheyaa Shree
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
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Jayashree Kadirvelu
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
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Hannah Lail
3Department of Nutrition, Georgia State University, Atlanta 30303, Georgia
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Desiree Wanders
3Department of Nutrition, Georgia State University, Atlanta 30303, Georgia
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Anne Z. Murphy
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
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Angela M. Mabb
1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
2Center for Behavioral Neuroscience, Georgia State University, Atlanta 30303, Georgia
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Article Information

DOI 
https://doi.org/10.1523/ENEURO.0074-23.2023
PubMed 
38164552
Published By 
Society for Neuroscience
History 
  • Received March 5, 2023
  • Revision received October 10, 2023
  • Accepted November 6, 2023
  • Published online December 27, 2023.
Copyright & Usage 
Copyright © 2024 George et al. This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International license, which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

Author Information

  1. Arlene J. George1,2,
  2. Wei Wei1,2,
  3. Dhanya N. Pyaram1,2,
  4. Morgan Gomez1,
  5. Nitheyaa Shree1,
  6. Jayashree Kadirvelu1,
  7. Hannah Lail3,
  8. Desiree Wanders3,
  9. Anne Z. Murphy1 and
  10. Angela M. Mabb1,2
  1. 1Neuroscience Institute, Georgia State University, Atlanta 30302, Georgia
  2. 2Center for Behavioral Neuroscience, Georgia State University, Atlanta 30303, Georgia
  3. 3Department of Nutrition, Georgia State University, Atlanta 30303, Georgia
  1. Correspondence should be addressed to Angela M. Mabb at amabb{at}gsu.edu.
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Author contributions

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

Disclosures

  • The authors declare no competing financial interests.

  • We acknowledge Zachary Allen, for assistance in the development, genotyping, and maintenance of the Rnf216 KO mice and Hannah Harder for guidance and sharing code for statistical analysis of microglia. This work was funded by the National Institute of Neurological Disorders and Stroke (grant R21NS116760) and National Science Foundation Career Award (2047700), National Ataxia Foundation Young Investigator Research Grant, the Cleon C. Arrington Research Initiation Grant Program (RIG-93), and a Molecular Basis of Disease Seed Grant to A.M.M.; A.Z.M. is funded by the National Institutes of Health (DA041529); A.J.G., M.G., and H.L. were funded by the Brains & Behavior Fellowship. A.J.G. was funded by the Kenneth W. and Georganne F. Honeycutt Fellowship.

Funding

  • NIH/NINDS

    R21NS116760
  • National Ataxia Foundation Young Investigator Research Grant

  • Cleon C. Arrington Research Initiation Grant Program

    RIG-93
  • Molecular Basis of Disease Grant

  • NIH

    DA041529
  • Brains & Behavior Fellowship

  • Kenneth W. and Georganne F. Honeycutt Fellowship

  • National Science Foundation (NSF)

    2047700

Other Version

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Gordon Holmes Syndrome Model Mice Exhibit Alterations in Microglia, Age, and Sex-Specific Disruptions in Cognitive and Proprioceptive Function
Arlene J. George, Wei Wei, Dhanya N. Pyaram, Morgan Gomez, Nitheyaa Shree, Jayashree Kadirvelu, Hannah Lail, Desiree Wanders, Anne Z. Murphy, Angela M. Mabb
eNeuro 27 December 2023, 11 (1) ENEURO.0074-23.2023; DOI: 10.1523/ENEURO.0074-23.2023

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Gordon Holmes Syndrome Model Mice Exhibit Alterations in Microglia, Age, and Sex-Specific Disruptions in Cognitive and Proprioceptive Function
Arlene J. George, Wei Wei, Dhanya N. Pyaram, Morgan Gomez, Nitheyaa Shree, Jayashree Kadirvelu, Hannah Lail, Desiree Wanders, Anne Z. Murphy, Angela M. Mabb
eNeuro 27 December 2023, 11 (1) ENEURO.0074-23.2023; DOI: 10.1523/ENEURO.0074-23.2023
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Keywords

  • ataxia
  • cognitive
  • Gordon Holmes syndrome
  • microglia
  • RNF216
  • ubiquitin

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