Go to JCI Insight
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
  • Clinical Research and Public Health
  • Current issue
  • Past issues
  • By specialty
    • COVID-19
    • Cardiology
    • Gastroenterology
    • Immunology
    • Metabolism
    • Nephrology
    • Neuroscience
    • Oncology
    • Pulmonology
    • Vascular biology
    • All ...
  • Videos
    • Conversations with Giants in Medicine
    • Video Abstracts
  • Reviews
    • View all reviews ...
    • Pancreatic Cancer (Jul 2025)
    • Complement Biology and Therapeutics (May 2025)
    • Evolving insights into MASLD and MASH pathogenesis and treatment (Apr 2025)
    • Microbiome in Health and Disease (Feb 2025)
    • Substance Use Disorders (Oct 2024)
    • Clonal Hematopoiesis (Oct 2024)
    • Sex Differences in Medicine (Sep 2024)
    • View all review series ...
  • Viewpoint
  • Collections
    • In-Press Preview
    • Clinical Research and Public Health
    • Research Letters
    • Letters to the Editor
    • Editorials
    • Commentaries
    • Editor's notes
    • Reviews
    • Viewpoints
    • 100th anniversary
    • Top read articles

  • Current issue
  • Past issues
  • Specialties
  • Reviews
  • Review series
  • Conversations with Giants in Medicine
  • Video Abstracts
  • In-Press Preview
  • Clinical Research and Public Health
  • Research Letters
  • Letters to the Editor
  • Editorials
  • Commentaries
  • Editor's notes
  • Reviews
  • Viewpoints
  • 100th anniversary
  • Top read articles
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
Top
  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal
  • Top
  • Footnotes
  • Version history
  • Article usage
  • Citations to this article

Advertisement

Corrigendum Free access | 10.1172/JCI147307

Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model

Yueqin Zhou, Sharon Carmona, A.K.M.G. Muhammad, Shaughn Bell, Jesse Landeros, Michael Vazquez, Ritchie Ho, Antonietta Franco, Bin Lu, Gerald W. Dorn II, Shaomei Wang, Cathleen M. Lutz, and Robert H. Baloh

Find articles by Zhou, Y. in: PubMed | Google Scholar

Find articles by Carmona, S. in: PubMed | Google Scholar

Find articles by Muhammad, A. in: PubMed | Google Scholar

Find articles by Bell, S. in: PubMed | Google Scholar

Find articles by Landeros, J. in: PubMed | Google Scholar

Find articles by Vazquez, M. in: PubMed | Google Scholar

Find articles by Ho, R. in: PubMed | Google Scholar

Find articles by Franco, A. in: PubMed | Google Scholar

Find articles by Lu, B. in: PubMed | Google Scholar

Find articles by Dorn, G. in: PubMed | Google Scholar

Find articles by Wang, S. in: PubMed | Google Scholar

Find articles by Lutz, C. in: PubMed | Google Scholar

Find articles by Baloh, R. in: PubMed | Google Scholar

Published January 19, 2021 - More info

Published in Volume 131, Issue 2 on January 19, 2021
J Clin Invest. 2021;131(2):e147307. https://doi.org/10.1172/JCI147307.
© 2021 American Society for Clinical Investigation
Published January 19, 2021 - Version history
View PDF

Related article:

Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model
Yueqin Zhou, … , Cathleen M. Lutz, Robert H. Baloh
Yueqin Zhou, … , Cathleen M. Lutz, Robert H. Baloh
Research Article Neuroscience

Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model

  • Text
  • PDF
Abstract

Mitofusin-2 (MFN2) is a mitochondrial outer-membrane protein that plays a pivotal role in mitochondrial dynamics in most tissues, yet mutations in MFN2, which cause Charcot-Marie-Tooth disease type 2A (CMT2A), primarily affect the nervous system. We generated a transgenic mouse model of CMT2A that developed severe early onset vision loss and neurological deficits, axonal degeneration without cell body loss, and cytoplasmic and axonal accumulations of fragmented mitochondria. While mitochondrial aggregates were labeled for mitophagy, mutant MFN2 did not inhibit Parkin-mediated degradation, but instead had a dominant negative effect on mitochondrial fusion only when MFN1 was at low levels, as occurs in neurons. Finally, using a transgenic approach, we found that augmenting the level of MFN1 in the nervous system in vivo rescued all phenotypes in mutant MFN2R94Q-expressing mice. These data demonstrate that the MFN1/MFN2 ratio is a key determinant of tissue specificity in CMT2A and indicate that augmentation of MFN1 in the nervous system is a viable therapeutic strategy for the disease.

Authors

Yueqin Zhou, Sharon Carmona, A.K.M.G. Muhammad, Shaughn Bell, Jesse Landeros, Michael Vazquez, Ritchie Ho, Antonietta Franco, Bin Lu, Gerald W. Dorn II, Shaomei Wang, Cathleen M. Lutz, Robert H. Baloh

×

Original citation: J Clin Invest. 2019;129(4):1756–1771. https://doi.org/10.1172/JCI124194

Citation for this corrigendum: J Clin Invest. 2021;131(2):e147307. https://doi.org/10.1172/JCI147307

The forward primer sequence for genotyping the founder mice was incorrect. The correct sentence is below. In addition, the sequence has been corrected online.

Primers were as follows: forward, 5′-TCTGAGTGGCAAAGGACCTTAGG; reverse, 5′-TCCAGTTCTGCATTCCTGTACG.

The authors regret the error.

Footnotes

See the related article at Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model.

Version history
  • Version 1 (January 19, 2021): Electronic publication

Article tools

  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal

Metrics

  • Article usage
  • Citations to this article

Go to

  • Top
  • Footnotes
  • Version history
Advertisement
Advertisement

Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

Sign up for email alerts