Histological bulbar manifestations in the ALS rat

Osama N. Kashlan, Bader N. Kashlan, Sang Su Oh, Lisa M. McGinley, Kevin S. Chen, Robbi Kupfer, Audrey B Erman, Stacey A. Sakowski, Eva L. Feldman

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Almost all patients with amyotrophic lateral sclerosis (ALS) develop bulbar symptoms; therefore, it is important to have valid animal models that accurately reflect these features. While the SOD1-G93A rat is extensively used as an ALS model, bulbar symptoms in this model are not well characterized. Objective: In the present study, we aimed to better characterize bulbar dysfunction in terms of histology to determine whether the SOD1-G93A rat is a useful model for bulbar-onset ALS. Methods: Sixty-day-old SOD1-G93A rats on a Sprague-Dawley background and age-matched wild-type controls were assessed weekly for global motor function, facial nerve function, and vagal nerve function. The study endpoint was determined when an SOD1-G93A rat could not right itself within 30 s of being placed on its side. At that point, neuronal counts were assessed in different brainstem cranial nerve nuclei. In addition, the masseter muscle, posterior belly of the digastric muscle, and tongue muscle were evaluated for intact neuromuscular junctions. Results: Our data demonstrate decreases in the number of motor neurons in the trigeminal, facial, and hypoglossal nuclei, as well as compromised neuromuscular junction integrity in the muscles they innervate. Conclusion: These findings suggest that, from a histological standpoint, the SOD1-G93A rat is a valid model of ALS bulbar symptoms.

Original languageEnglish (US)
Pages (from-to)121-126
Number of pages6
JournalNeurodegenerative Diseases
Volume15
Issue number2
DOIs
StatePublished - May 6 2015
Externally publishedYes

Fingerprint

Amyotrophic Lateral Sclerosis
Neuromuscular Junction
Muscles
Masseter Muscle
Cranial Nerves
Facial Nerve
Motor Neurons
Tongue
Brain Stem
Histology
Animal Models

Keywords

  • Bulbar amyotrophic lateral sclerosis
  • Cranial nerve function
  • Cu<sup>2+</sup>/Zn<sup>2+</sup> superoxide dismutase
  • Motor function
  • Neuromuscular junction
  • Rat model of amyotrophic lateral sclerosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Kashlan, O. N., Kashlan, B. N., Oh, S. S., McGinley, L. M., Chen, K. S., Kupfer, R., ... Feldman, E. L. (2015). Histological bulbar manifestations in the ALS rat. Neurodegenerative Diseases, 15(2), 121-126. https://doi.org/10.1159/000377725

Histological bulbar manifestations in the ALS rat. / Kashlan, Osama N.; Kashlan, Bader N.; Oh, Sang Su; McGinley, Lisa M.; Chen, Kevin S.; Kupfer, Robbi; Erman, Audrey B; Sakowski, Stacey A.; Feldman, Eva L.

In: Neurodegenerative Diseases, Vol. 15, No. 2, 06.05.2015, p. 121-126.

Research output: Contribution to journalArticle

Kashlan, ON, Kashlan, BN, Oh, SS, McGinley, LM, Chen, KS, Kupfer, R, Erman, AB, Sakowski, SA & Feldman, EL 2015, 'Histological bulbar manifestations in the ALS rat', Neurodegenerative Diseases, vol. 15, no. 2, pp. 121-126. https://doi.org/10.1159/000377725
Kashlan ON, Kashlan BN, Oh SS, McGinley LM, Chen KS, Kupfer R et al. Histological bulbar manifestations in the ALS rat. Neurodegenerative Diseases. 2015 May 6;15(2):121-126. https://doi.org/10.1159/000377725
Kashlan, Osama N. ; Kashlan, Bader N. ; Oh, Sang Su ; McGinley, Lisa M. ; Chen, Kevin S. ; Kupfer, Robbi ; Erman, Audrey B ; Sakowski, Stacey A. ; Feldman, Eva L. / Histological bulbar manifestations in the ALS rat. In: Neurodegenerative Diseases. 2015 ; Vol. 15, No. 2. pp. 121-126.
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