Abstract
Background ALS is a fatal, rapidly progressive neurodegenerative disorder affecting motor neurons in the CNS; this results in muscle weakness which progresses to paralysis and death from respiratory failure. There is currently no effective cure as its pathophysiology is poorly understood; however, aggregates comprising misfolded proteins are known to be characteristic of the disease. These protein aggregations could elicit ER stress and subsequently the unfolded protein response (UPR). Initially, this response is cytoprotective as it inhibits protein synthesis thereby preventing further protein accumulation until the stress resolves, however if prolonged it can stimulate apoptosis. This study attempts to clarify the role of ER stress and the UPR in ALS.
Highlights
ALS is a fatal, rapidly progressive neurodegenerative disorder affecting motor neurons in the CNS; this results in muscle weakness which progresses to paralysis and death from respiratory failure
ER stress was shown to be increased in the SOD1 mouse model compared to controls; these mice are a model for ALS as 20% familial ALS cases carry the same mutation
Co-localisation of the NeuN with the p-eif2a implies a neuronal location of the stress. This indicates the unfolded protein response (UPR)’s involvement in the pathophysiology of ALS and suggests it may be a delayed response or a consequence of the disease as markers were exceedingly elevated in the endstage compared to the day 90 mouse spinal cords
Summary
ALS is a fatal, rapidly progressive neurodegenerative disorder affecting motor neurons in the CNS; this results in muscle weakness which progresses to paralysis and death from respiratory failure. There is currently no effective cure as its pathophysiology is poorly understood; aggregates comprising misfolded proteins are known to be characteristic of the disease. These protein aggregations could elicit ER stress and subsequently the unfolded protein response (UPR). This response is cytoprotective as it inhibits protein synthesis thereby preventing further protein accumulation until the stress resolves, if prolonged it can stimulate apoptosis. This study attempts to clarify the role of ER stress and the UPR in ALS
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