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Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease).

Abstract
Pathological studies of mice homozygous for the motor neuron degeneration (Mnd) mutation show abnormalities similar to those of the human neuronal ceroid lipofuscinoses: sudanophilic, autofluorescent intraneuronal inclusions that are immunoreactive with antibodies to subunit c of mitochondrial ATP synthase. Ultrastructurally, the inclusions have the pentalaminar structure characteristic of some form of human neuronal ceroid lipofuscinosis and of canine and ovine models of neuronal ceroid lipofuscinosis. Similar inclusions are observed in many somatic organs and in the retina, which develops photoreceptor degeneration. This mutation, previously considered a model of amyotrophic lateral sclerosis, may be a useful model for molecular and genetic studies of human neuronal ceroid lipofuscinosis because mice have been well characterized genetically. Since they are inexpensive to breed and maintain, they can also be used to test therapeutic interventions.
AuthorsR T Bronson, B D Lake, S Cook, S Taylor, M T Davisson
JournalAnnals of neurology (Ann Neurol) Vol. 33 Issue 4 Pg. 381-5 (Apr 1993) ISSN: 0364-5134 [Print] United States
PMID7683855 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Indoles
  • Luxol Fast Blue MBS
Topics
  • Animals
  • Disease Models, Animal
  • Immunohistochemistry
  • Inclusion Bodies (ultrastructure)
  • Indoles
  • Mice
  • Mice, Inbred C57BL
  • Motor Neuron Disease (pathology, veterinary)
  • Neuronal Ceroid-Lipofuscinoses (pathology)
  • Neurons (ultrastructure)
  • Rodent Diseases (pathology)
  • Staining and Labeling

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