scholarly journals Alterations in the motor neuron-renshaw cell circuit in the Sod1 G93A mouse model

2013 ◽  
Vol 521 (7) ◽  
pp. Spc1-Spc1
Author(s):  
Hanna Wootz ◽  
Eileen FitzSimons-Kantamneni ◽  
Martin Larhammar ◽  
Travis M. Rotterman ◽  
Anders Enjin ◽  
...  
Keyword(s):  
Mouse Model ◽  
Motor Neuron ◽  
Renshaw Cell ◽  
Cell Circuit ◽  
Sod1 G93a Mouse

scholarly journals Alterations in the motor neuron-renshaw cell circuit in theSod1G93Amouse model

2013 ◽  
Vol 521 (7) ◽  
pp. 1449-1469 ◽  
Author(s):  
Hanna Wootz ◽  
Eileen FitzSimons-Kantamneni ◽  
Martin Larhammar ◽  
Travis M. Rotterman ◽  
Anders Enjin ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Renshaw Cell ◽  
Cell Circuit

scholarly journals Microarray Analysis of the Cellular Pathways Involved in the Adaptation to and Progression of Motor Neuron Injury in the SOD1 G93A Mouse Model of Familial ALS

2007 ◽  
Vol 27 (34) ◽  
pp. 9201-9219 ◽  
Author(s):  
L. Ferraiuolo ◽  
P. R. Heath ◽  
H. Holden ◽  
P. Kasher ◽  
J. Kirby ◽  
...  
Keyword(s):  
Mouse Model ◽  
Motor Neuron ◽  
Microarray Analysis ◽  
Familial Als ◽  
Cellular Pathways ◽  
Neuron Injury ◽  
Sod1 G93a Mouse ◽  
Motor Neuron Injury

scholarly journals Novel P2X7 Antagonist Ameliorates the Early Phase of ALS Disease and Decreases Inflammation and Autophagy in SOD1-G93A Mouse Model

2021 ◽  
Vol 22 (19) ◽  
pp. 10649
Author(s):  
Savina Apolloni ◽  
Paola Fabbrizio ◽  
Susanna Amadio ◽  
Giulia Napoli ◽  
Mattia Freschi ◽  
...  
Keyword(s):  
Mouse Model ◽  
Motor Neuron ◽  
Disease Progression ◽  
P2x7 Receptor ◽  
The Novel ◽  
Motor Neuron Survival ◽  
Anti Inflammatory ◽  
Lateral Sclerosis ◽  
Sod1 G93a Mouse

Amyotrophic lateral sclerosis (ALS) is a disease with a resilient neuroinflammatory component caused by activated microglia and infiltrated immune cells. How to successfully balance neuroprotective versus neurotoxic actions through the use of anti-inflammatory agents is still under debate. There has been a boost of awareness regarding the role of extracellular ATP and purinergic receptors in modulating the physiological and pathological mechanisms in the nervous system. Particularly in ALS, it is known that the purinergic ionotropic P2X7 receptor plays a dual role in disease progression by acting at different cellular and molecular levels. In this context, we previously demonstrated that the P2X7 receptor antagonist, brilliant blue G, reduces neuroinflammation and ameliorates some of the pathological features of ALS in the SOD1-G93A mouse model. Here, we test the novel, noncommercially available, and centrally permeant Axxam proprietary P2X7 antagonist, AXX71, in SOD1-G93A mice, by assessing some behavioral and molecular parameters, among which are disease progression, survival, gliosis, and motor neuron wealth. We demonstrate that AXX71 affects the early symptomatic phase of the disease by reducing microglia-related proinflammatory markers and autophagy without affecting the anti-inflammatory markers or motor neuron survival. Our results suggest that P2X7 modulation can be further investigated as a therapeutic strategy in preclinical studies, and exploited in ALS clinical trials.

In vitroactivation of GAT1 transporters expressed in spinal cord gliosomes stimulates glutamate release that is abnormally elevated in the SOD1/G93A(+) mouse model of amyotrophic lateral sclerosis

2010 ◽  
Vol 113 (2) ◽  
pp. 489-501 ◽  
Author(s):  
Marco Milanese ◽  
Simona Zappettini ◽  
Emanuela Jacchetti ◽  
Tiziana Bonifacino ◽  
Chiara Cervetto ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Glutamate Release ◽  
Lateral Sclerosis ◽  
Sod1 G93a Mouse

scholarly journals Verapamil Ameliorates Motor Neuron Degeneration and Improves Lifespan in the SOD1G93A Mouse Model of ALS by Enhancing Autophagic Flux

2019 ◽  
Vol 10 (6) ◽  
pp. 1159 ◽  
Author(s):  
Xiaojie Zhang ◽  
Sheng Chen ◽  
Kaili Lu ◽  
Feng Wang ◽  
Jiangshan Deng ◽  
...  
Keyword(s):  
Mouse Model ◽  
Motor Neuron ◽  
Autophagic Flux ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Sod1g93a Mouse

scholarly journals Novel behavioural characteristics of the superoxide dismutase 1 G93A ( SOD1 G93A ) mouse model of amyotrophic lateral sclerosis include sex‐dependent phenotypes

2019 ◽  
Vol 19 (2) ◽  
Author(s):  
Fabian Kreilaus ◽  
Stefan Guerra ◽  
Rebecca Masanetz ◽  
Victoria Menne ◽  
Justin Yerbury ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Superoxide Dismutase ◽  
Mouse Model ◽  
Superoxide Dismutase 1 ◽  
Behavioural Characteristics ◽  
G93a Sod1 ◽  
Lateral Sclerosis ◽  
Sod1 G93a Mouse

Survival motor neuron deficiency enhances progression in an amyotrophic lateral sclerosis mouse model

2009 ◽  
Vol 34 (3) ◽  
pp. 511-517 ◽  
Author(s):  
Bradley J. Turner ◽  
Nicholas J. Parkinson ◽  
Kay E. Davies ◽  
Kevin Talbot
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Survival Motor Neuron ◽  
Lateral Sclerosis
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