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Lack of habituation in somatosensory cortex but not in visual cortex of ALS patients.
Amyotroph Lateral Scler Frontotemporal Degener
November 2024
Faculty of Medicine, Department of Neurology, Hacettepe University, Ankara, Turkey.
Objective: Amyotrophic lateral sclerosis (ALS) is a multisystem degenerative disease with extra-motor components. In ALS, there is also hyperexcitability of extra-motor areas. Habituation is defined as ''a response decrement" caused by repeated stimulations.
View Article and Find Full Text PDFHow is Excitotoxicity Being Modelled in iPSC-Derived Neurons?
Neurotox Res
October 2024
Wicking Dementia Research and Education Centre, College of Health and Medicine, University of Tasmania, 17 Liverpool Street, Hobart, TAS, Australia.
Article Synopsis
- Excitotoxicity, linked to environmental factors and neuronal hyperexcitability, is a key mechanism in the neurodegeneration seen in ALS (amyotrophic lateral sclerosis).
- Animal models and advances in induced pluripotent stem cell (iPSC) technologies have allowed researchers to study excitotoxic mechanisms in a more human-relevant context, facilitating the exploration of gene-environment interactions.
- The review emphasizes the importance of understanding neurotransmitter receptor expressions in iPSC-derived neurons, as well as new methods for inducing and studying excitotoxicity to better grasp the pathological processes involved in ALS.
Unlabelled: Neuronal hyperexcitability is a hallmark of amyotrophic lateral sclerosis (ALS) but its relationship with the TDP-43 aggregates that comprise the predominant pathology in over 90% of ALS cases remains unclear. Emerging evidence in tissue and slice culture models indicate that TDP-43 pathology induces neuronal hyperexcitability suggesting it may be responsible for the excitotoxicity long believed to be a major driver of ALS neuron death. Here, we characterized hyperexcitability and neurodegeneration in the hippocampus of doxycycline-regulatable rNLS8 mice (NEFH-tTA x tetO-hTDP-43ΔNLS), followed by treatment with AAV encoded DREADDs and anti-seizure medications to measure the effect on behavioral function and neurodegeneration.
View Article and Find Full Text PDFDirect interrogation of cortical interneuron circuits in amyotrophic lateral sclerosis.
Brain
October 2024
The Brain and Nerve Research Centre, Concord Clinical School, The University of Sydney, NSW, 2139, Australia.
Article Synopsis
- Cortical hyperexcitability in amyotrophic lateral sclerosis (ALS) is linked to complex interactions between cortical interneurons, with this study examining GABA-ergic dysfunction via a combination of transcranial magnetic stimulation (TMS) and EEG.
- In 21 ALS patients, TMS-EEG results showed significant differences in the transcranial evoked potential (TEP) components compared to healthy controls, indicating impaired cortical inhibitory function.
- Notably, changes in TEP components correlated with muscle weakness and longer disease duration, highlighting the role of disrupted GABA-ergic circuits in the progression of ALS.
Structural basis for the rescue of hyperexcitable cells by the amyotrophic lateral sclerosis drug Riluzole.
Nat Commun
September 2024
School of Natural Sciences, Birkbeck University of London, London, United Kingdom.
Article Synopsis
- * The ALS drug riluzole works by stabilizing VGSCs in their inactivated state, which suppresses excessive late sodium current (I) and reduces cellular overexcitability.
- * Riluzole's mechanism involves entering the VGSCs through special openings in their structure, allowing for targeted inhibition of I without blocking sodium conduction, which could guide future drug design and potential repurposing of riluzole.
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