Article
A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease.
Neuron - 1 Oct 1994
Lee M K, Marszalek J R, Cleveland D W
Abstract excerpt
A direct role of aberrant neurofilament accumulation in the etiology of human motor neuron diseases, including amyotrophic lateral sclerosis, is suggested by the presence of abnormal accumulations of neurofilaments as an early hallmark of the pathogenic process. Furthermore, forcing increased expression of neurofilament subunits in transgenic mouse models leads to motor neuron dysfunction, albeit without the...
Topics
- Amino Acid Sequence
- Animals
- Axons
- Base Sequence
- Cell Death
- Humans
- Mice
- Mice, Transgenic
- Microscopy, Electron
- Molecular Sequence Data
- Motor Neuron Disease