Neuronal intermediate filaments consist of the NFL subunit linked with NFM and NFH, and their alterations have been proposed as a pathogenesic cause in motor neuron diseases. Depletion of the Nefl gene in mice mimicks the reduced NFL mRNA levels seen in amyotrophic lateral sclerosis and causes perik
Mice with the deleted neurofilament of low molecular weight (Nefl) gene: 2. Effects on motor functions and spatial orientation
โ Scribed by M. Dubois; C. Strazielle; J.-P. Julien; R. Lalonde
- Publisher
- John Wiley and Sons
- Year
- 2005
- Tongue
- English
- Weight
- 104 KB
- Volume
- 80
- Category
- Article
- ISSN
- 0360-4012
No coin nor oath required. For personal study only.
โฆ Synopsis
Mice with a null mutation of the Nefl gene were compared with normal controls in tests of motor activity, equilibrium, and spatial orientation. Despite a normal capacity to ambulate, NFL -/- mice had fewer rears in an open field, crossed fewer segments on stationary beams, and fell more frequently when suspended on a horizontal bar. In addition, the distance swum before reaching the escape platform was greater in NFL -/- mice than in controls during acquisition of place learning in the Morris water maze at the start of training. The motor impairments were linearly correlated with increased cytochrome oxidase activity seen in cerebellum and brainstem. These results indicate that, as early as 6 months, depletion of the NFL protein is sufficient to cause mild sensorimotor dysfunctions and spatial deficits, but without overt signs of paresis.
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