Skip to main content
construction release_alert
Scholars@Duke will be undergoing maintenance April 11-15. Some features may be unavailable during this time.
cancel

Motor axonopathies in a mouse model of Duchenne muscular dystrophy.

Publication ,  Journal Article
Dhindsa, JS; McCall, AL; Strickland, LM; Fusco, AF; Kahn, AF; ElMallah, MK
Published in: Sci Rep
June 2, 2020

Duchenne muscular dystrophy (DMD) is a fatal neuromuscular disease caused by deleterious mutations in the DMD gene which encodes the dystrophin protein. Skeletal muscle weakness and eventual muscle degradation due to loss of dystrophin are well-documented pathological hallmarks of DMD. In contrast, the neuropathology of this disease remains understudied despite the emerging evidence of neurological abnormalities induced by dystrophin loss. Using quantitative morphological analysis of nerve sections, we characterize axonopathies in the phrenic and hypoglossal (XII) nerves of mdx mice. We observe dysfunction in these nerves - which innervate the diaphragm and genioglossus respectively - that we propose contributes to respiratory failure, the most common cause of death in DMD. These observations highlight the importance in the further characterization of the neuropathology of DMD. Additionally, these observations underscore the necessity in correcting both the nervous system pathology in addition to skeletal muscle deficits to ameliorate this disease.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Sci Rep

DOI

EISSN

2045-2322

Publication Date

June 2, 2020

Volume

10

Issue

1

Start / End Page

8967

Location

England

Related Subject Headings

  • Respiratory Insufficiency
  • Phrenic Nerve
  • Muscular Dystrophy, Duchenne
  • Mice, Inbred mdx
  • Loss of Function Mutation
  • Hypoglossal Nerve
  • Dystrophin
  • Disease Models, Animal
  • Diaphragm
  • Axons
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Dhindsa, J. S., McCall, A. L., Strickland, L. M., Fusco, A. F., Kahn, A. F., & ElMallah, M. K. (2020). Motor axonopathies in a mouse model of Duchenne muscular dystrophy. Sci Rep, 10(1), 8967. https://doi.org/10.1038/s41598-020-65824-1
Dhindsa, Justin S., Angela L. McCall, Laura M. Strickland, Anna F. Fusco, Amanda F. Kahn, and Mai K. ElMallah. “Motor axonopathies in a mouse model of Duchenne muscular dystrophy.Sci Rep 10, no. 1 (June 2, 2020): 8967. https://doi.org/10.1038/s41598-020-65824-1.
Dhindsa JS, McCall AL, Strickland LM, Fusco AF, Kahn AF, ElMallah MK. Motor axonopathies in a mouse model of Duchenne muscular dystrophy. Sci Rep. 2020 Jun 2;10(1):8967.
Dhindsa, Justin S., et al. “Motor axonopathies in a mouse model of Duchenne muscular dystrophy.Sci Rep, vol. 10, no. 1, June 2020, p. 8967. Pubmed, doi:10.1038/s41598-020-65824-1.
Dhindsa JS, McCall AL, Strickland LM, Fusco AF, Kahn AF, ElMallah MK. Motor axonopathies in a mouse model of Duchenne muscular dystrophy. Sci Rep. 2020 Jun 2;10(1):8967.

Published In

Sci Rep

DOI

EISSN

2045-2322

Publication Date

June 2, 2020

Volume

10

Issue

1

Start / End Page

8967

Location

England

Related Subject Headings

  • Respiratory Insufficiency
  • Phrenic Nerve
  • Muscular Dystrophy, Duchenne
  • Mice, Inbred mdx
  • Loss of Function Mutation
  • Hypoglossal Nerve
  • Dystrophin
  • Disease Models, Animal
  • Diaphragm
  • Axons