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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<jats:italic>superoxide dismutase 1<\/jats:italic>(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<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mrow><mml:msup><mml:mrow><mml:mtext>SOD<\/mml:mtext><mml:mn>1<\/mml:mn><\/mml:mrow><mml:mrow><mml:mtext>G<\/mml:mtext><mml:mn>93<\/mml:mn><mml:mtext>A<\/mml:mtext><\/mml:mrow><\/mml:msup><\/mml:mrow><\/mml:math>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.<\/jats:p>","DOI":"10.1155\/2014\/852163","type":"journal-article","created":{"date-parts":[[2014,4,27]],"date-time":"2014-04-27T21:04:09Z","timestamp":1398632649000},"page":"1-13","source":"Crossref","is-referenced-by-count":40,"title":["Accumulation of Misfolded SOD1 in Dorsal Root Ganglion Degenerating Proprioceptive Sensory Neurons of Transgenic Mice with Amyotrophic Lateral Sclerosis"],"prefix":"10.1155","volume":"2014","author":[{"given":"Javier","family":"S\u00e1bado","sequence":"first","affiliation":[{"name":"Unitat de Neurobiologia Cellular, Departament de Medicina Experimental, Facultat de Medicina, IRBLLEIDA, Universitat de Lleida and Institut de Recerca Biom\u00e8dica de Lleida, Avenida Rovira Roure 80, Lleida, 25198 Catalonia, 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Departament de Medicina Experimental, Facultat de Medicina, IRBLLEIDA, Universitat de Lleida and Institut de Recerca Biom\u00e8dica de Lleida, Avenida Rovira Roure 80, Lleida, 25198 Catalonia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00eddia","family":"Piedrafita","sequence":"additional","affiliation":[{"name":"Unitat de Neurobiologia Cellular, Departament de Medicina Experimental, Facultat de Medicina, IRBLLEIDA, Universitat de Lleida and Institut de Recerca Biom\u00e8dica de Lleida, Avenida Rovira Roure 80, Lleida, 25198 Catalonia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Calder\u00f3","sequence":"additional","affiliation":[{"name":"Unitat de Neurobiologia Cellular, Departament de Medicina Experimental, Facultat de Medicina, IRBLLEIDA, Universitat de Lleida and Institut de Recerca Biom\u00e8dica de Lleida, Avenida Rovira Roure 80, Lleida, 25198 Catalonia, 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