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  • Title: Accelerated and widespread neuronal loss occurs in motor neuron degeneration (mnd) mice expressing a neurofilament-disrupting transgene.
    Author: Plummer J, Peterson A, Messer A.
    Journal: Mol Cell Neurosci; 1995 Dec; 6(6):532-43. PubMed ID: 8742270.
    Abstract:
    To examine the effects of multiple stressors on the onset and specificity of a neurodegenerative disease, we derived mnd/mnd mice expressing a neurofilament-H/lacZ transgene. The mnd mutation causes adult-onset motor dysfunction, and produces abnormal ubiquitous accumulation of autofluorescent lipopigment, with retinal degeneration and late-onset motor neuron degeneration. The neurofilament H-beta-galactosidase fusion protein causes endogenous neurofilament subunits to precipitate in perikarya, but shows neither significant neuronal degeneration nor behavioral changes until advanced age. In mnd/mnd-transgenic animals, neurological symptoms, lipopigment accumulation, and motor neuron loss were substantially accelerated. Newly vulnerable populations of neurons also degenerated, including cerebellar Purkinje cells and dorsal roots. This study exemplifies a synergistic interaction between a neuron-specific and a ubiquitous defect, leading to significant neurological consequences. It further indicates that cytoskeletal abnormalities similar to those observed in late-onset human neurodegenerative disorders can interact with other cellular defects and contribute to pathogenesis.
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