Cytoskeletal abnormalities have been reported in cases of amyotrophic lateral sclerosis
(ALS) including abnormal inclusions containing neurofilaments (NFs) and/or peripherin,
reduced mRNA levels for the NF light (NF-L) protein and mutations in the NF heavy (NF-H)
gene. Recently, transgenic mouse approaches have been used to address whether
cytoskeletal changes may contribute to motor neuron disease. Mice lacking one of the three
NF subunits are viable and do not develop motor neuron disease. Nonetheless, mice with …