Results 31 to 40 of about 247,506 (355)
Advances in motor neurone disease [PDF]
Journal of the Royal Society of Medicine, 2014 Summary Motor neurone disease (MND), the commonest clinical presentation of which is amyotrophic lateral sclerosis (ALS), is regarded as the most devastating of adult-onset neurodegenerative disorders. The last decade has seen major improvements in patient care, but also rapid scientific advances, so that rational therapies based on key pathogenic ...
Bäumer, D, Talbot, K, Turner, M
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Protective potential of glucagon like peptide 2 (GLP-2) against the neurodegeneration [PDF]
, 2019 Neurodegeneration consists in loss of neuron specific types, pattern and distribution, leading to progressive dysfunctions of the central nervous system.
Amato, Antonella, Mulè, Flavia
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Neuroimaging of motor neuron diseases [PDF]
Therapeutic Advances in Neurological Disorders, 2012 It is agreed that conventional magnetic resonance imaging (MRI) of the brain and spine is one of the core elements in the differential diagnostic work up of patients with clinical signs of motor neuron diseases (MNDs), for example amyotrophic lateral sclerosis (ALS), to exclude MND mimics. However, the sensitivity and specificity of MRI signs in these
Jan Kassubek +2 more
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Cell Reports, 2013 Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by a deficiency in the survival motor neuron (SMN) protein. SMN mediates the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs) and possibly other RNPs.
Sarah Tisdale +7 more
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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
Nature Communications, 2016 The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a ...
Aarti Sharma +9 more
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The Role of DNA Damage in Neural Plasticity in Physiology and Neurodegeneration
Frontiers in Cellular Neuroscience, 2022 Damage to DNA is generally considered to be a harmful process associated with aging and aging-related disorders such as neurodegenerative diseases that involve the selective death of specific groups of neurons.
Anna Konopka +2 more
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Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy [PDF]
, 2016 Spinal muscular atrophy (SMA) is a neuromuscular disease caused by low levels of SMN protein, primarily affecting lower motor neurons. Recent evidence from SMA and related conditions suggests that glial cells can influence disease severity.
Brophy, Peter J. +9 more
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DNA Damage, Defective DNA Repair, and Neurodegeneration in Amyotrophic Lateral Sclerosis
Frontiers in Aging Neuroscience, 2022 DNA is under constant attack from both endogenous and exogenous sources, and when damaged, specific cellular signalling pathways respond, collectively termed the “DNA damage response.” Efficient DNA repair processes are essential for cellular viability ...
Anna Konopka +2 more
doaj +1 more source
UBA1/GARS-dependent pathways drive sensory-motor connectivity defects in spinal muscular atrophy [PDF]
, 2018 Deafferentation of motor neurons as a result of defective sensory-motor connectivity is a critical early event in the pathogenesis of spinal muscular atrophy, but the underlying molecular pathways remain unknown.
Boyd, Penelope J. +10 more
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Biology Open, 2013 Summary Amyotrophic Lateral Sclerosis (ALS) is a motor neuron degenerative disease characterized by a progressive, and ultimately fatal, muscle paralysis. The human VAMP-Associated Protein B (hVAPB) is the causative gene of ALS type 8.
Mario Sanhueza +3 more
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