Amyotrophic lateral sclerosis (ALS) is an adult-onset neurological disorder characterized by
the selective loss of motor neurons. A pathological hallmark of both sporadic and familial
ALS is the presence of abnormal accumulations of neurofilament and peripherin proteins in
motor neurons. In the past decade, transgenic mouse approaches have been used to
address the role of such cytoskeletal abnormalities in motor neuron disease and also to
unravel the pathogenesis caused by mutations in the gene coding for superoxide dismutase …