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Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress
- Fabiola Rojas, N. Cortés, Sebastián Abarzúa, A. Dyrda, B. van Zundert
- BiologyFront. Cell. Neurosci.
- 3 October 2013
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.
Trehalose delays the progression of amyotrophic lateral sclerosis by enhancing autophagy in motoneurons
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.
Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling
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.
Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability.
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.
αVβ3 Integrin regulates astrocyte reactivity
- Raúl Lagos-Cabré, A. Alvarez, L. Leyton
- Biology, MedicineJournal of Neuroinflammation
- 29 September 2017
Therefore, inflammation induces expression of αVβ3 Integrin and other proteins, astrocyte reactivity, and Thy-1 responsiveness, and ectopic control of β3 integrin levels modulates these responses regardless of inflammation.
Chronic infusion of SOD1G93A astrocyte‐secreted factors induces spinal motoneuron degeneration and neuromuscular dysfunction in healthy rats
- U. N. Ramírez-Jarquín, Fabiola Rojas, B. van Zundert, R. Tapia
- BiologyJournal of cellular physiology
- 1 October 2017
It is demonstrated that factors released in vitro from astrocytes derived from ALS mice cause spinal motoneuron death and consequent neuromuscular dysfunction in vivo.
Widespread loss of the silencing epigenetic mark H3K9me3 in astrocytes and neurons along with hippocampal-dependent cognitive impairment in C9orf72 BAC transgenic mice
The data indicate that a loss of the repressive mark H3K9me3 in astrocytes and neurons in the central nervous system of C9BAC mice represents a signature during neurodegeneration and memory deficit of C 9ALS/FTD.
Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons
Mutant Sod1-expressing Astrocytes Release Toxic Factors That Trigger Motor Neuron Death
It is suggested that riluzole, the only FDA-approved drug with known benefits for ALS patients, acts by inhibiting hyper-excitability, an observation with direct implications for therapy of ALS.