Pathophysiological and diagnostic implications of cortical dysfunction in ALS

N Geevasinga, P Menon, PH Özdinler… - Nature Reviews …, 2016 - nature.com
Cortical dysfunction—specifically, the development of hyperexcitability—seems to be an
early and intrinsic feature of sporadic and familial amyotrophic lateral sclerosis (ALS) …

Microglia centered pathogenesis in ALS: insights in cell interconnectivity

D Brites, AR Vaz - Frontiers in cellular neuroscience, 2014 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is the most common and most aggressive form of adult
motor neuron (MN) degeneration. The cause of the disease is still unknown, but some …

Protein misfolding, aggregation and mechanism of amyloid cytotoxicity: An overview and therapeutic strategies to inhibit aggregation

M Zaman, AN Khan, SM Zakariya, RH Khan - International journal of …, 2019 - Elsevier
Protein and peptides are converted from their soluble forms into highly ordered fibrillar
aggregates under various conditions inside the cell. Such transitions confer diverse …

Targeting NMDA receptors at the neurovascular unit: Past and future treatments for central nervous system diseases

C Seillier, F Lesept, O Toutirais, F Potzeha… - International journal of …, 2022 - mdpi.com
The excitatory neurotransmission of the central nervous system (CNS) mainly involves
glutamate and its receptors, especially N-methyl-D-Aspartate receptors (NMDARs). These …

Sources and triggers of oxidative damage in neurodegeneration

PR Angelova - Free Radical Biology and Medicine, 2021 - Elsevier
Neurodegeneration describes a group of more than 300 neurological diseases,
characterised by neuronal loss and intra-or extracellular protein depositions, as key …

Building a bridge between NMDAR-mediated excitotoxicity and mitochondrial dysfunction in chronic and acute diseases

RG Mira, W Cerpa - Cellular and Molecular Neurobiology, 2021 - Springer
Glutamate is the major excitatory neurotransmitter in the brain, and it is widely accepted to
play a role in synaptic plasticity and excitotoxic cell death. Glutamate binds to several …

Cortical hyperexcitability: Diagnostic and pathogenic biomarker of ALS

S Vucic, N Pavey, M Haidar, BJ Turner, MC Kiernan - Neuroscience letters, 2021 - Elsevier
Cortical hyperexcitability is an early and intrinsic feature of both sporadic and familial forms
of amyotrophic lateral sclerosis (ALS).. Importantly, cortical hyperexcitability appears to be …

Synaptic dysfunction and altered excitability in C9ORF72 ALS/FTD

A Starr, R Sattler - Brain research, 2018 - Elsevier
Amyotrophic lateral sclerosis (ALS) is characterized by a progressive degeneration of upper
and lower motor neurons, resulting in fatal paralysis due to denervation of the muscle. Due …

Driven to decay: excitability and synaptic abnormalities in amyotrophic lateral sclerosis

MJ Fogarty - Brain research bulletin, 2018 - Elsevier
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron (MN) disease and is
clinically characterised by the death of corticospinal motor neurons (CSMNs), spinal and …

Altered functionality, morphology, and vesicular glutamate transporter expression of cortical motor neurons from a presymptomatic mouse model of amyotrophic lateral …

L Saba, MT Viscomi, S Caioli, A Pignataro… - Cerebral …, 2016 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a lethal disorder characterized by the gradual
degeneration of motor neurons in the cerebrospinal axis. Whether upper motor neuron …