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Mutant SOD1 accumulation in sensory neurons does not associate with endoplasmic reticulum stress features: Implications for differential vulnerability of sensory and motor neurons to SOD1 toxicity
- Publication Year :
- 2016
- Publisher :
- Elsevier Ireland Ltd, 2016.
-
Abstract
- Mutations in Cu/Zn-superoxide dismutase (SOD1) cause familial amyotrophic lateral sclerosis (ALS). Previous papers showed that mutant SOD1 accumulates and undergoes misfolding in motor neurons and that the specific interaction of mutant SOD1 with derlin-1 leads to endoplasmic reticulum (ER) stress and activation of the unfolded protein response (UPR). Because evidence shows that mutant SOD1 expression also damages sensory neurons, we hypothesized that, similarly to motor neurons, the sensory neurons of ALS mouse model SOD1(G93A) accumulate mutant/misfolded SOD1 and suffer from ER stress and UPR activation. Our results reveal that SOD1(G93A) sensory neurons accumulate mutant/misfolded SOD1 but, surprisingly, do not suffer from ER stress and UPR activation. Moreover, the sensory neurons do not express detectable levels of the SOD1 interactor derlin-1. These results suggest a potential molecular mechanism underlying the differential vulnerability of motor and sensory neurons to mutant SOD1 toxicity.
- Subjects :
- 0301 basic medicine
Sensory Receptor Cells
animal diseases
Mutant
SOD1
Sensory system
Mice, Transgenic
Biology
medicine.disease_cause
Motor Neuron
Sensory Receptor Cell
Unfolded protein response
03 medical and health sciences
Mice
0302 clinical medicine
Superoxide Dismutase-1
Ganglia, Spinal
medicine
Endoplasmic reticulum stre
Animals
Humans
Amyotrophic lateral sclerosis
Membrane Protein
Amyotrophic lateral sclerosi
Motor Neurons
Mutation
Neuroscience (all)
Animal
General Neuroscience
Endoplasmic reticulum
Amyotrophic Lateral Sclerosis
Membrane Proteins
nutritional and metabolic diseases
Endoplasmic Reticulum Stress
medicine.disease
nervous system diseases
Dorsal root ganglion neuron
Disease Models, Animal
030104 developmental biology
nervous system
Unfolded Protein Response
Neuroscience
030217 neurology & neurosurgery
Human
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....fd1c877d40ac726cc08b949e367679f0