The superoxide dismutase1 (SOD1) G93A mutation does not promote neuronal injury after focal brain ischemia and optic nerve transection in mice.
The superoxide dismutase 1 (SOD1)G93A mouse was recently established as transgenic model of amyotrophic lateral sclerosis. We were interested to know whether the SOD1 G93A mutation promotes neuronal injury after intraluminal middle cerebral artery thread occlusion and/or retinal ganglion cell (RGC) axotomy in mice, which are highly reproducible and clinically relevant in vivo models of acute and subacute neuronal degeneration, respectively. In our experiments, G93A mutant SOD1 neither influenced ischemic injury after 90 or 30 min of focal ischemia, nor had an impact on the severity of RGC degeneration after optic nerve transection, when human SOD1 G93A mutant mice were compared to human wild-type SOD1 mice. Our data indicate that the clinically relevant SOD1 G93A mutation, which leads to amyotrophic lateral sclerosis in humans and mice, does not necessarily worsen neuronal degeneration in other pathologies. Thus, the G93A mutation may be counterbalanced in non-motor neurons of young animals, and region-specific and age-related factors may be necessary so that neurodegeneration is re-enforced.
Kilic
E
E
Department of Neurology, University Hospital Zürich, Frauenklinikstrasse 26, CH-8091, Switzerland. ertugrul.kilic@usz.ch
Weishaupt
J H
JH
Kilic
U
U
Rohde
G
G
Yulug
B
B
Peters
K
K
Hermann
D M
DM
Bähr
M
M
D000818:Animals; D002545:Brain Ischemia / Q000378:metabolism / Q000473:pathology*; D002470:Cell Survival; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D020221:Optic Nerve Injuries / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D012165:Retinal Ganglion Cells / Q000378:metabolism; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism*; D000072105:Superoxide Dismutase-1
C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1