scholarly journals Early-Stage Treatment with Withaferin A Reduces Levels of Misfolded Superoxide Dismutase 1 and Extends Lifespan in a Mouse Model of Amyotrophic Lateral Sclerosis

2014 ◽  
Vol 12 (1) ◽  
pp. 217-233 ◽  
Author(s):  
Priyanka Patel ◽  
Jean-Pierre Julien ◽  
Jasna Kriz
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Early Stage ◽  
Withaferin A ◽  
Superoxide Dismutase 1 ◽  
Lateral Sclerosis

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.

Overexpression of Aβ is associated with acceleration of onset of motor impairment and superoxide dismutase 1 aggregation in an amyotrophic lateral sclerosis mouse model

Aging Cell ◽  
2006 ◽  
Vol 5 (2) ◽  
pp. 153-165 ◽  
Author(s):  
Qiao-Xin Li ◽  
Su San Mok ◽  
Katrina M. Laughton ◽  
Catriona A. McLean ◽  
Irene Volitakis ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Motor Impairment ◽  
Superoxide Dismutase 1 ◽  
Lateral Sclerosis

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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