title: Histological Bulbar Manifestations in the ALS Rat.
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.
  
  
  CONCLUSIONS
  These findings suggest that, from a histological standpoint, the SOD1-G93A rat is a valid model of ALS bulbar symptoms.
authors.affiliation: Department of Neurosurgery, University of Michigan, Ann Arbor, Mich., USA.
authors.forename: Osama N
authors.initials: ON
authors.lastname: Kashlan
authors.forename: Bader N
authors.initials: BN
authors.lastname: Kashlan
authors.forename: Sang Su
authors.initials: SS
authors.lastname: Oh
authors.forename: Lisa M
authors.initials: LM
authors.lastname: McGinley
authors.forename: Kevin S
authors.initials: KS
authors.lastname: Chen
authors.forename: Robbi
authors.initials: R
authors.lastname: Kupfer
authors.forename: Audrey
authors.initials: A
authors.lastname: Erman
authors.forename: Stacey A
authors.initials: SA
authors.lastname: Sakowski
authors.forename: Eva L
authors.initials: EL
authors.lastname: Feldman
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology*; D000818:Animals; D001835:Body Weight / Q000235:genetics; D001933:Brain Stem / Q000473:pathology*; D004195:Disease Models, Animal; D008297:Male; D009046:Motor Neurons / Q000473:pathology*; D009469:Neuromuscular Junction / Q000235:genetics / Q000473:pathology; D051381:Rats; D017207:Rats, Sprague-Dawley; D055647:Rats, Transgenic; D013482:Superoxide Dismutase / Q000235:genetics; D014059:Tongue / Q000473:pathology
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase