Results 41 to 50 of about 167,403 (289)
Amyotrophic Lateral Sclerosis.
Internal Medicine, 1995 The final cascade of amyotrophic lateral sclerosis (ALS) coincides with the onset of clinical neurological deficits and involves multifactorial interactive mechanisms. These terminal events include excitotoxicity, free radical accumulation and possibly immunological disturbances.
openaire +2 more sources
Cervical spinal cord atrophy in amyotrophic lateral sclerosis across disease stages
Annals of Clinical and Translational Neurology, 2023 Objective Spinal cord degeneration is a hallmark of amyotrophic lateral sclerosis. The assessment of gray matter and white matter cervical spinal cord atrophy across clinical stages defined using the King's staging system could advance the understanding ...
Anna Nigri +12 more
doaj +1 more source
FEBS Letters, EarlyView.We identified a systemic, progressive loss of protein S‐glutathionylation—detected by nonreducing western blotting—alongside dysregulation of glutathione‐cycle enzymes in both neuronal and peripheral tissues of Taiwanese SMA mice. These alterations were partially rescued by SMN antisense oligonucleotide therapy, revealing persistent redox imbalance as ...
Sofia Vrettou, Brunhilde Wirth
wiley +1 more source
The extracellular domain of neurotrophin receptor p75 as a candidate biomarker for amyotrophic lateral sclerosis. [PDF]
PLoS ONE, 2014 Objective biomarkers for amyotrophic lateral sclerosis would facilitate the discovery of new treatments. The common neurotrophin receptor p75 is up regulated and the extracellular domain cleaved from injured neurons and peripheral glia in amyotrophic ...
Stephanie R Shepheard +4 more
doaj +1 more source
Adaptation to G93A superoxide dismutase 1 in a motor neuron cell line model of amyotrophic lateral sclerosis. The role of glutathione [PDF]
, 2009 Motor neuron degeneration in amyotrophic lateral sclerosis involves oxidative damage. Glutathione (GSH) is critical as an antioxidant and a redox modulator.
AND LAVINIA CANTONI +5 more
core +1 more source
FEBS Letters, EarlyView.Biomolecular condensates formed by fused in sarcoma (FUS) are dissolved by high ATP concentrations yet persist in cells. Using a reconstituted system, we demonstrate that valosin‐containing protein (VCP), an AAA+ ATPase, counteracts ATP‐driven dissolution of FUS condensates through its D2 ATPase activity.
Hitomi Kimura +2 more
wiley +1 more source
Data in Brief, 2020 A standardised, single-centre, longitudinal imaging protocol was used to evaluate longitudinal brainstem alterations in 100 patients with amyotrophic lateral sclerosis (ALS) with reference to 33 patients with primary lateral sclerosis (PLS), 30 patients ...
Peter Bede +11 more
doaj +1 more source
The Compound Muscle Action Potential as Neurophysiological Marker for Amyotrophic Lateral Sclerosis. [PDF]
, 2016 Objectives: To definite the peripheral nervous involvement in ALS through the repeated use of the compound motor action potential (CMAP) to test the progression of disease, to determine different change of phrenic CMAP and forced vital capacity (FVC) in
CECCANTI , MARCO +7 more
core
Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration [PDF]
, 2018 Mutations in Cu/Zn superoxide dismutase 1 (SOD1) lead to Amyotrophic Lateral Sclerosis (ALS), a neurodegenerative disease that disproportionately affects glutamatergic and cholinergic motor neurons.
Baskoylu, Saba N +10 more
core +3 more sources
FEBS Open Bio, EarlyView.This study investigated a novel WST‐8‐based assay for evaluating d‐Amino acid oxidase (DAO) inhibitors. We confirmed its effectiveness using known inhibitors and found that uremic toxins possess relatively weak inhibitory activity compared to existing drugs.
Kahoko Miyake +4 more
wiley +1 more source