Abstract
Fig4 null reduces phosphatidylinositol-3,5-diphosphate concentration and causes severe
neuronal degeneration in both pale-tremor (plt) mice and patients with Charcot-Marie-Tooth disease type 4J (CMT4J), an inherited
condition with recessive mutations in FIG4. Our previous study shows that minor trauma is associated with an accelerated course
of motor neuron degeneration in patients with CMT4J. Heterozygous loss of FIG4 function has been suggested to be a risk factor in developing sporadic amyotrophic
lateral sclerosis. We therefore hypothesize that minor trauma may trigger or exacerbate
motor neuron degeneration in mice with fig4 haploinsufficiency (plt+/−). We have studied 18 wild-type and 18 plt+/− mice and created nerve injury by compressing the sciatic nerve. Outcomes in the
mice were evaluated by nerve conduction study, Rotarod, and nerve morphology. No differences
were found between wild-type and plt+/− mice. Taken together, our results demonstrate that haploinsufficiency of fig4 does not impose risks in rodents to develop neuronal degeneration in either naïve
or traumatic conditions.
Keywords
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Article info
Publication history
Published online: August 29, 2011
Accepted:
August 4,
2011
Received in revised form:
July 9,
2011
Received:
January 22,
2011
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
