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@ARTICLE{Tessitore:285015,
author = {Tessitore, Sara and Torazza, Carola and Bonifacino, Tiziana
and Bacchetti, Francesca and Roselli, Francesco and Raiteri,
Luca and Milanese, Marco and Bonanno, Giambattista},
title = {{F}ocus on the excitatory and inhibitory neurotransmission
imbalance in amyotrophic lateral sclerosis: a harmful
disease player or a potential therapeutic opportunity?},
journal = {Neurobiology of disease},
volume = {219},
issn = {0969-9961},
address = {[Amsterdam]},
publisher = {Elsevier},
reportid = {DZNE-2026-00141},
pages = {107272},
year = {2026},
abstract = {Amyotrophic lateral sclerosis (ALS) is a progressive and
fatal neurodegenerative disease affecting both upper and
lower motor neurons. Evidence indicates that ALS is a
'multifactorial' and 'multicellular' disease; however, the
causes of ALS remain elusive, as the mechanisms underlying
the disease have not yet been completely clarified. One
major proposed mechanism, first described in 1990, is the
glutamate excitotoxicity theory. This theory suggests that
excessive glutamatergic neurotransmission, combined with
impaired glutamate clearance, significantly contributes to
motor neuron degeneration. Aberrant glutamate
neurotransmission may lead to precocious motor neuron
hyperexcitability in the brain cortex and spinal cord, which
can be later followed by hypoexcitability phases.
Accumulating evidence suggests that impairment in inhibitory
neurotransmission is relevant for excitation/inhibition
imbalance, leading to excitotoxicity, a critical feature of
ALS. Gamma-aminobutyric acid (GABA) and glycine are the
primary inhibitory neurotransmitters that modulate neuronal
excitability, including that of motor neurons. In ALS,
dysfunction of inhibitory processes and loss of cortical and
spinal inhibitory interneurons are observed. Renshaw cells,
which mediate recurrent inhibition in the spinal cord, seem
particularly vulnerable. The interactions among
neurotransmitters, including glutamate, GABA, and glycine,
play pivotal roles in regulating the excitation/inhibition
balance. Auto- or hetero-receptor-mediated interactions are
crucial, but auto- or hetero-transporter-mediated
neurotransmission control, as well as other molecular
mechanisms that regulate neuronal interplay, are also
relevant, as they can be altered in pathological conditions
such as ALS. To facilitate the search for new effective
therapies for ALS, attention toward the impairment of
inhibitory neurotransmission is essential to determine the
role of excitation/inhibition imbalance on excitotoxicity.
Different pharmacological agents are being used to treat
other pathologies in which the excitation/inhibition ratio
is impaired. Among these, we highlighted the potential of
novel glycine and GABA receptor ligands and transporter
inhibitors, as stand-alone interventions or in combination
with other treatments. The present review aims to elucidate
the complex interplay between excitatory and inhibitory
neurotransmission in ALS, exploring the potential to target
this imbalance for therapeutic purposes.},
subtyp = {Review Article},
keywords = {Amyotrophic Lateral Sclerosis: physiopathology /
Amyotrophic Lateral Sclerosis: metabolism / Amyotrophic
Lateral Sclerosis: drug therapy / Humans / Synaptic
Transmission: physiology / Animals / Motor Neurons:
metabolism / Motor Neurons: physiology / Glutamic Acid:
metabolism / Neural Inhibition: physiology / Amyotrophic
lateral sclerosis (Other) / Cortex (Other) /
Excitation/inhibition balance (Other) / GABA (Other) /
Glutamate (Other) / Glycine (Other) / Ion channels (Other) /
Receptors (Other) / Spinal cord (Other) / Transporters
(Other) / Glutamic Acid (NLM Chemicals)},
cin = {AG Roselli},
ddc = {570},
cid = {I:(DE-2719)1910001},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41525888},
doi = {10.1016/j.nbd.2026.107272},
url = {https://pub.dzne.de/record/285015},
}