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ALS is a fatal disease of the motor neuron system leading to steadily progressive muscle weakness.
This can be expected in cases of development of supranuclear palsy or with predominant involvement of the upper motor neuron system, as suggested by our initial study 63.
A recent report described the propagation of TDP-43 pathology in ALS, which starts from the UMN and LMN systems and spreads to the anteromedial temporal lobes through the motor neuron system.
In a village in the Enshi prefecture whose inhabitants had a high prevalence of Se toxicosis from naturally occurring Se, motor neuron system abnormalities including paralysis and hemiplegia were noted.
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Two investigators (YR and MY) evaluated the degeneration of the motor neuron systems and the immunohistochemical profiles of the included patients.
With respect to the degeneration of motor neuron systems, the severity of motor neuron loss in the primary motor cortex, facial and hypoglossal nuclei and spinal anterior horns, myelin pallor within the CST and aggregation of macrophages within the primary motor cortex and CST were evaluated.
In ALS, the selective death of upper and lower motor neurons of the cortico-motor-neuronal system results in progressive and terminal atrophy of muscle.
Likewise, the motor neuron would render the system's "decision".
It presents an extensive description of novel motor neuron and peripheral nervous system defects in Fz3 mutants.
To further investigate the role of ADAR2 and AMPA receptors in motor neuron death, the Cre/loxP system was used to conditionally target the ADAR2 gene in the motor neurons of mice [ 23].
Furthermore, impairment of the ubiquitin proteasome system, but not the autophagy lysosome system, in motor neuron replicates ALS in mice (Tashiro et al, 2012).
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