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 …

Motoneuron excitability dysfunction in ALS: Pseudo‐mystery or authentic conundrum?

SM Elbasiouny - The Journal of physiology, 2022 - Wiley Online Library
In amyotrophic lateral sclerosis (ALS), abnormalities in motoneuronal excitability are seen in
early pathogenesis and throughout disease progression. Fully understanding motoneuron …

The vulnerability of spinal motoneurons and soma size plasticity in a mouse model of amyotrophic lateral sclerosis

SS Dukkipati, TL Garrett… - The Journal of …, 2018 - Wiley Online Library
Key points Motoneuron soma size is a largely plastic property that is altered during
amyotrophic lateral sclerosis (ALS) progression. We report evidence of systematic spinal …

Sigma 1 receptor as a therapeutic target for amyotrophic lateral sclerosis

M Herrando‐Grabulosa… - British journal of …, 2021 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is an adult disease causing a progressive loss of upper
and lower motoneurons, muscle paralysis and early death. ALS has a poor prognosis of 3–5 …

How degeneration of cells surrounding motoneurons contributes to amyotrophic lateral sclerosis

R Crabé, F Aimond, P Gosset, F Scamps, C Raoul - Cells, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by the
progressive degeneration of upper and lower motoneurons. Despite motoneuron death …

Accumulation of misfolded SOD1 outlines distinct patterns of motor neuron pathology and death during disease progression in a SOD1G93A mouse model of …

S Salvany, A Casanovas, L Piedrafita, S Gras… - Brain …, 2022 - Wiley Online Library
Early misfolded superoxide dismutase 1 (mfSOD1) accumulation, motor neuron (MN)
degeneration, and microgliosis are hallmark pathological features in SOD1G93A …

Decreased spinal cord motor neuron numbers in mice depleted of central nervous system copper

JR Liddell, JBW Hilton, YJ Wang, JL Billings… - Metallomics, 2024 - academic.oup.com
Disrupted copper availability in the central nervous system (CNS) is implicated as a
significant feature of the neurodegenerative disease amyotrophic lateral sclerosis (ALS) …

Excitatory and inhibitory neuronal circuits in the spinal cord and their role in the control of motor neuron function and degeneration

UN Ramírez-Jarquín, R Tapia - ACS Chemical Neuroscience, 2018 - ACS Publications
The complex neuronal networks of the spinal cord coordinate a wide variety of motor
functions, including walking, running, and voluntary and involuntary movements. This is …

Cytoplasmic TDP-43 accumulation drives changes in C-bouton number and size in a mouse model of sporadic Amyotrophic Lateral Sclerosis

AN Bak, S Djukic, M Kadlecova, TH Braunstein… - Molecular and Cellular …, 2023 - Elsevier
An altered neuronal excitability of spinal motoneurones has consistently been implicated in
Amyotrophic Lateral Sclerosis (ALS) leading to several investigations of synaptic input to …

Increased axon initial segment length results in increased Na+ currents in spinal motoneurones at symptom onset in the G127X SOD1 mouse model of amyotrophic …

HS Jørgensen, DB Jensen, KP Dimintiyanova… - Neuroscience, 2021 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease preferentially affecting
motoneurones. Transgenic mouse models have been used to investigate the role of …