A simple test to monitor the motor dysfunction in a transgenic mouse model of amyotrophic lateral sclerosis.
We reported recently that transgenic mice overexpressing human neurofilament heavy (NF-H) proteins develop a progressive neurological disorder with pathological features resembling those found in amyotrophic lateral sclerosis (ALS) (Côté et al 1993). A simple behavioral test, the grasping ability, has been used here for evaluating the motor dysfunction during aging of NF-H transgenic mice. Transgenic mice overexpressing NF-H proteins are normal at birth but they progressively fail to uphold their weight when tested for their grasping ability. The deficits in motor function during aging correlate with a progressive disruption of peripheral nerve function as evidenced by the atrophy and degeneration of distal axons.
Collard
J F
JF
Centre for Research in Neurosciences, McGill University, Montreal, Quebec, Canada.
Julien
J P
JP
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000473:pathology; D000818:Animals; D001369:Axons / Q000473:pathology; D005260:Female; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000473:pathology; D009048:Motor Skills / Q000502:physiology*; D016900:Neurofilament Proteins / Q000235:genetics; D010525:Peripheral Nerves / Q000473:pathology
D016900:Neurofilament Proteins; C051774:neurofilament protein H