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Neonatal Neuronal Circuitry Shows Hyperexcitable Disturbance in a Mouse Model of the Adult-Onset Neurodegenerative Disease Amyotrophic Lateral Sclerosis
TLDR
These findings underscore the widespread and early onset of abnormal neural activity in this mouse model of the adult neurodegenerative disease ALS, and suggest that suppression of PCNa and hyperexcitability early in life might be one way to mitigate or prevent cell death in the adult CNS. Expand
ALS‐linked protein disulfide isomerase variants cause motor dysfunction
TLDR
This study functionally characterized four ALS‐linked mutations recently identified in two major PDI genes, PDIA1 and PDIA3/ERp57, and identified ER proteostasis imbalance as a risk factor for ALS, driving initial stages of the disease. Expand
Differential roles of NMDA Receptor Subtypes NR2A and NR2B in dendritic branch development and requirement of RasGRF1.
TLDR
The association between NR1NR2B-receptors and RasGRF1 is needed for dendritic branch formation in VSCNs and hippocampal neurons in vitro and the dominated NR2A expression and the limited interactions of this subunit with the signaling protein Ras GRF1 may contribute to the restricted dendrite arbor development in the adult CNS. Expand
Alterations in the motor neuron–renshaw cell circuit in the Sod1G93A mouse model
TLDR
It is concluded that the loss of presynaptic motor axon input on Renshaw cells occurs at early stages of ALS and disconnects the recurrent inhibitory circuit, presumably resulting in diminished control of motor neuron firing. Expand
Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability.
TLDR
It is suggested that riluzole, the only FDA-approved drug with known benefits for ALS patients, acts by inhibiting hyperexcitability, a critical element mediating the non-cell-autonomous toxicity of ACM-hSOD1(G93A) on motoneurons is increased excitability. Expand
Vitamin C Is an Essential Antioxidant That Enhances Survival of Oxidatively Stressed Human Vascular Endothelial Cells in the Presence of a Vast Molar Excess of Glutathione*
TLDR
In endothelial cells under oxidative challenge, vitamin C functions as an essential cellular antioxidant even in the presence of a vast molar excess of glutathione. Expand
Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress
TLDR
It is shown that two completely unrelated ALS models lead to the death of motoneuron via non-cell-autonomous processes, and that astrocytes expressing mutations in SOD1 and TDP43 trigger such cell death through a common pathogenic pathway that involves nitroxidative stress, induced at least in part by Nav channel activity. Expand
Interactive and additive influences of Gender, BMI and Apolipoprotein 4 on cognition in children chronically exposed to high concentrations of PM2.5 and ozone. APOE 4 females are at highest risk in
TLDR
Gender, BMI and APOE influence children's cognitive responses to air pollution and glucose is likely a key player, and young female results highlight the urgent need for gender-targeted health programmes to improve cognitive responses. Expand
Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons
TLDR
Trehalose treatment led to a significant upregulation in the expression of key autophagy-related genes at the mRNA level including Lc3, Becn1, Sqstm1 and Atg5, and trehalose administration enhanced the nuclear translocation of FOXO1, an important transcription factor involved in the activation of autophagic in neurons. Expand
Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling
TLDR
A sequence of events in which a toxic factor(s) released by ALS-expressing astrocytes rapidly induces hyper-excitability, which in turn increases calcium influx and affects mitochondrial structure and physiology is examined. Expand
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