title: 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.
authors.affiliation: Jackson Laboratory, Bar Harbor, ME 04609-0800.
authors.forename: R T
authors.initials: RT
authors.lastname: Bronson
authors.forename: B D
authors.initials: BD
authors.lastname: Lake
authors.forename: S
authors.initials: S
authors.lastname: Cook
authors.forename: S
authors.initials: S
authors.lastname: Taylor
authors.forename: M T
authors.initials: MT
authors.lastname: Davisson
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure; D007211:Indoles; D051379:Mice; D008810:Mice, Inbred C57BL; D016472:Motor Neuron Disease / Q000473:pathology / Q000662:veterinary*; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology*; D009474:Neurons / Q000648:ultrastructure; D012376:Rodent Diseases / Q000473:pathology*; D013194:Staining and Labeling
chemical_list: D007211:Indoles; C018588:Luxol Fast Blue MBS