Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis
Joint Authors
Esquerda, Josep E.
Casanovas, Anna
Tarabal, Olga
Sábado, Javier
Hereu, Marta
Piedrafita, Lídia
Calderó, Jordi
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-13, 13 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-04-27
Country of Publication
Egypt
No. of Pages
13
Main Subjects
Abstract EN
Amyotrophic lateral sclerosis (ALS) is an adult-onset progressive neurodegenerative disease affecting upper and lower motoneurons (MNs).
Although the motor phenotype is a hallmark for ALS, there is increasing evidence that systems other than the efferent MN system can be involved.
Mutations of superoxide dismutase 1 (SOD1) gene cause a proportion of familial forms of this disease.
Misfolding and aggregation of mutant SOD1 exert neurotoxicity in a noncell autonomous manner, as evidenced in studies using transgenic mouse models.
Here, we used the SOD1G93A mouse model for ALS to detect, by means of conformational-specific anti-SOD1 antibodies, whether misfolded SOD1-mediated neurotoxicity extended to neuronal types other than MNs.
We report that large dorsal root ganglion (DRG) proprioceptive neurons accumulate misfolded SOD1 and suffer a degenerative process involving the inflammatory recruitment of macrophagic cells.
Degenerating sensory axons were also detected in association with activated microglial cells in the spinal cord dorsal horn of diseased animals.
As large proprioceptive DRG neurons project monosynaptically to ventral horn MNs, we hypothesise that a prion-like mechanism may be responsible for the transsynaptic propagation of SOD1 misfolding from ventral horn MNs to DRG sensory neurons.
American Psychological Association (APA)
Sábado, Javier& Casanovas, Anna& Tarabal, Olga& Hereu, Marta& Piedrafita, Lídia& Calderó, Jordi…[et al.]. 2014. Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis. BioMed Research International،Vol. 2014, no. 2014, pp.1-13.
https://search.emarefa.net/detail/BIM-503390
Modern Language Association (MLA)
Sábado, Javier…[et al.]. Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis. BioMed Research International No. 2014 (2014), pp.1-13.
https://search.emarefa.net/detail/BIM-503390
American Medical Association (AMA)
Sábado, Javier& Casanovas, Anna& Tarabal, Olga& Hereu, Marta& Piedrafita, Lídia& Calderó, Jordi…[et al.]. Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis. BioMed Research International. 2014. Vol. 2014, no. 2014, pp.1-13.
https://search.emarefa.net/detail/BIM-503390
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-503390