Animal models of ALS

Erik P. Pioro, Hiroshi Mitsumoto*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

46 Scopus citations

Abstract

Animal models of amyotrophic lateral sclerosis (ALS) provide a unique opportunity to study this incurable and fatal human disease both clinically and pathologically. This is particularly true for certain pathological and therapeutic studies that are impractical or impossible to perform in human patients. Nonetheless, postmortem ALS tissue remains the 'gold standard' against which pathologic findings in animal models must be compared. Four natural disease models have been most extensively studied, including three mouse models: motor neuron degeneration (Mnd), progressive motor neuronopathy (pmn), wobbler, and one canine model: hereditary, canine spinal muscular atrophy (HCSMA). The wobbler mouse has been the most extensively studied of these models with analyses of clinical, pathological (perikaryon, axon, muscle), and biochemical features. Experimentally induced ALS animal models have allowed controlled testing of various neurotoxic, vital and immune- mediated mechanisms. Molecular techniques have recently generated mouse models in which genes relevant to the human disease or motor neuron biology have been manipulated. The most clinically relevant of these is a transgenic mouse overexpressing the mutated SOD1 gene of FALS patients, which has already provided significant insights into mechanisms of motor neuron degeneration in this disease. Because no single animal model perfectly reflects all the clinical and pathological characteristics of ALS, study of selected features from the most relevant models will contribute to a better understanding of the pathogenesis and/or etiology of this disease.

Original languageEnglish (US)
Pages (from-to)375-385
Number of pages11
JournalClinical Neuroscience
Volume3
Issue number6
StatePublished - 1995
Externally publishedYes

Keywords

  • Cu,Zn superoxide dismutase (SOD1)
  • gene knockout
  • hereditary canine spinal muscular atrophy (HCSMA)
  • motor neuron degeneration (Mnd)
  • neurotoxic
  • progressive motor neuronopathy (pmn)
  • transgenic mouse
  • viral
  • wobbler

ASJC Scopus subject areas

  • Neuroscience(all)
  • Neuropsychology and Physiological Psychology

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