The effect of peripheral nerve injury on disease progression in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis
Section snippets
Experimental procedures
Transgenic mice with a G93A mutation of the human SOD1 gene (TgN[SOD1-G93A]1Gur; Jackson Laboratory, Bar Harbor, ME, USA), were maintained by breeding male heterozygous carriers to female B6SJLF1 hybrids. The SOD1(G93A) transgenic mice were identified using polymerase chain reaction amplification of the transgene from genomic DNA. Male mice were used in these experiments and all experimental procedures were carried out under Licence from the Home Office in accordance with The Animals
Assessment of disease progression in SOD1(G93A) mice
The decline in motor function and extent of motoneuron degeneration in SOD1(G93A) mice was assessed at the approximate time of disease onset, at 90 days of age, when there was behavioural signs of motor deficits such hindlimb tremor, and also at the end-stage of the disease when the animals were 130 days old.
Discussion
In this study we examined disease progression in a transgenic mouse model of familial ALS and assessed the effect of inducing motoneurons to grow, by injury to their peripheral nerve, on motoneuron survival and muscle function.
Acknowledgments
This work was supported by the Brain Research Trust (BRT). P.S. was in receipt of an MRC Studentship and L.G. is the Graham Watts Senior Research Fellow, funded by the BRT.
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