scholarly journals Intralingual Administration of AAVrh10-miRSOD1 Improves Respiratory But Not Swallowing Function in a Superoxide Dismutase-1 Mouse Model of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 31 (15-16) ◽  
pp. 828-838 ◽  
Author(s):  
Lori A. Lind ◽  
Ellyn M. Andel ◽  
Angela L. McCall ◽  
Justin S. Dhindsa ◽  
Katherine A. Johnson ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Superoxide Dismutase 1 ◽  
Swallowing Function ◽  
Lateral Sclerosis

scholarly journals Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis

eLife ◽  
2014 ◽  
Vol 3 ◽  
Author(s):  
Félix Leroy ◽  
Boris Lamotte d'Incamps ◽  
Rebecca D Imhoff-Manuel ◽  
Daniel Zytnicki
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Postnatal Development ◽  
Superoxide Dismutase 1 ◽  
Critical Test ◽  
Early Postnatal Development ◽  
Contracting Muscle ◽  
Lateral Sclerosis ◽  
Motoneuron Degeneration

In amyotrophic lateral sclerosis (ALS) the large motoneurons that innervate the fast-contracting muscle fibers (F-type motoneurons) are vulnerable and degenerate in adulthood. In contrast, the small motoneurons that innervate the slow-contracting fibers (S-type motoneurons) are resistant and do not degenerate. Intrinsic hyperexcitability of F-type motoneurons during early postnatal development has long been hypothesized to contribute to neural degeneration in the adult. Here, we performed a critical test of this hypothesis by recording from identified F- and S-type motoneurons in the superoxide dismutase-1 mutant G93A (mSOD1), a mouse model of ALS at a neonatal age when early pathophysiological changes are observed. Contrary to the standard hypothesis, excitability of F-type motoneurons was unchanged in the mutant mice. Surprisingly, the S-type motoneurons of mSDO1 mice did display intrinsic hyperexcitability (lower rheobase, hyperpolarized spiking threshold). As S-type motoneurons are resistant in ALS, we conclude that early intrinsic hyperexcitability does not contribute to motoneuron degeneration.

scholarly journals Restrictive Lung Disease in the Cu/Zn Superoxide-Dismutase 1 G93A Amyotrophic Lateral Sclerosis Mouse Model

2017 ◽  
Vol 56 (3) ◽  
pp. 405-408 ◽  
Author(s):  
Lorelei Stoica ◽  
Allison M. Keeler ◽  
Lang Xiong ◽  
Michael Kalfopoulos ◽  
Kaitlyn Desrochers ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Lung Disease ◽  
Superoxide Dismutase 1 ◽  
Restrictive Lung Disease ◽  
Lateral Sclerosis
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