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Our findings support the notion that accumulation of DNA damage and genotoxic stress may contribute to neuronal aging and motor neuron vulnerability in human neuromuscular disorders.
The data support the hypothesis that accumulation of DNA damage and genotoxic stress significantly influences neuronal aging processes and may contribute to motor neuron vulnerability in human neuromuscular disorders.
Ringer, C., Weihe, E. & Schutz, B. Calcitonin gene related peptide expression levels predict motor neuron vulnerability in the superoxide dismutase 1 G93A mouse model of amyotrophic lateral sclerosis.
This may also allow an overall proinflammatory profile with motor neuron vulnerability well recognised in SOD1 ALS mice25, 63 and human ALS, and could provide some answers as to why global suppression of inflammation has failed in human clinical trials.
In this study we were particularly keen to identify regulators of motor neuron vulnerability.
This creates a valuable experimental paradigm to investigate the factors that regulate motor neuron vulnerability.
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Early axonal vulnerability in motor neuron diseases is linked to axonal transport impairment [16], [34], [35].
These susceptibility factors may increase the vulnerability of motor neuron viability during disease.
These mice exhibited marked vulnerability to motor neuron loss after axonal injury [ 8].
Despite a range of causes and severities, motor neuron diseases are united by the common vulnerability of motor neurons.
In addition to their greater vulnerability to bacterial hemolysins, increased susceptibility of neurons expressing the G93A mutant in their SOD1 to the attack of activated immune cells may be the pathophysiological basis of the vulnerability of the nervous system of patients with motor neuron disease to systemic infections.
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Justyna Jupowicz-Kozak
CEO of Professional Science Editing for Scientists @ prosciediting.com