Results 21 to 30 of about 151,768 (312)

Motor neurone disease [PDF]

open access: yesBMJ, 1996
In patients with motor neurone disease, progressively worsening breathlessness often coincides with deteriorating bulbar function, and this combination may lead to difficulties in swallowing and coughing and a risk of aspiration. Occasionally, chest infections cause life threatening respiratory failure.
openaire   +2 more sources

Real-time visualization of oxidative stress-mediated neurodegeneration of individual spinal motor neurons in vivo

open access: yesRedox Biology, 2018
Generation of reactive oxygen species (ROS) has been shown to be important for many physiological processes, ranging from cell differentiation to apoptosis. With the development of the genetically encoded photosensitiser KillerRed (KR) it is now possible
Isabel Formella   +8 more
doaj   +1 more source

Neurons other than motor neurons in motor neuron disease.

open access: yesHistology and histopathology, 2017
Amyotrophic lateral sclerosis (ALS) is typically defined by a loss of motor neurons in the central nervous system. Accordingly, morphological analysis for decades considered motor neurons (in the cortex, brainstem and spinal cord) as the neuronal population selectively involved in ALS.
Ruffoli R   +7 more
openaire   +5 more sources

Inflammation in ALS and SMA: Sorting out the good from the evil

open access: yesNeurobiology of Disease, 2010
Indices of neuroinflammation are found in a variety of diseases of the CNS including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA).
Dimitra Papadimitriou   +5 more
doaj   +1 more source

Long-term muscle-specific overexpression of DOK7 in mice using AAV9-tMCK-DOK7

open access: yesMolecular Therapy: Nucleic Acids, 2023
Neuromuscular junction (NMJ) dysfunction underlies several diseases, including congenital myasthenic syndromes (CMSs) and motor neuron disease (MND).
Yu-Ting Huang   +8 more
doaj   +1 more source

Diagnosing motor neurone disease [PDF]

open access: yesBMJ, 1996
Advanced motor neurone disease is easily recognised. Progressively worsening dysphagia and dysarthria, and widespread wasting and weakness of the limbs with hyperactive reflexes in a corresponding wasted myotome in the absence of other neurological signs are unmistakable features.
openaire   +2 more sources

SMN Is Essential for the Biogenesis of U7 Small Nuclear Ribonucleoprotein and 3′-End Formation of Histone mRNAs

open access: yesCell 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
doaj   +1 more source

Equine Motor Neuron Disease

open access: yesVeterinary Clinics of North America: Equine Practice, 1997
This article reviews the subject of equine motor neuron disease, a neurodegenerative disease of horses. The authors discuss various topics, including epidemiology, pathophysiology, clinical signs, laboratory findings, diagnosis, and treatment.
T J, Divers   +2 more
openaire   +2 more sources

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

open access: yesNature 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
doaj   +1 more source

TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex

open access: yesNeurobiology of Disease, 2022
Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by the loss of upper and lower motor neurons in the brain and spinal cord.
Stephanie L. Rayner   +15 more
doaj   +1 more source

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