title: Involvement of sensory innervation in the skin of SOD1(G93A) ALS mice.
abstract: Sensory alterations have been described in both amyotrophic lateral sclerosis (ALS) patients and mouse models. While involvement of intraepidermal and subepidermal axons has been shown in skin biopsies of ALS patients, it is unclear if the SOD1(G93A) mouse presents similar alterations. We analyzed the epidermal and dermal innervation, based on PGP9.5 immunostaining, of SOD1(G93A) mice at different stages. The results showed a marked reduction of intraepidermal nerve fibers, Meissner's corpuscles, and subepidermal nerve density already at 4 weeks. This loss of innervation progressed over time. Dermal axonal density decreased at a later stage of the disease. There was a gradient of axonal loss, with a more severe decline in the epidermis compared with deeper structures, indicating a distal axonal neuropathy as the mechanism of degeneration. These findings suggest that the analysis of the cutaneous sensory innervation may be an accessible and useful tool to assess the neurodegeneration process in motoneuron diseases.
authors.affiliation: Neuromuscular Unit, Department of Neurology, Hospital del Mar, Barcelona, Spain.|Department of Cell Biology, Physiology and Immunology, Institute of Neurosciences and CIBERNED, Universitat Autònoma de Barcelona, Bellaterra, Spain.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Rubio
authors.affiliation: Department of Cell Biology, Physiology and Immunology, Institute of Neurosciences and CIBERNED, Universitat Autònoma de Barcelona, Bellaterra, Spain.
authors.forename: Mireia
authors.initials: M
authors.lastname: Herrando-Grabulosa
authors.affiliation: Department of Cell Biology, Physiology and Immunology, Institute of Neurosciences and CIBERNED, Universitat Autònoma de Barcelona, Bellaterra, Spain.
authors.forename: Jorge J
authors.initials: JJ
authors.lastname: Vilches
authors.affiliation: Department of Cell Biology, Physiology and Immunology, Institute of Neurosciences and CIBERNED, Universitat Autònoma de Barcelona, Bellaterra, Spain.
authors.forename: Xavier
authors.initials: X
authors.lastname: Navarro
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D000818:Animals; D004195:Disease Models, Animal; D005500:Follow-Up Studies; D008465:Mechanoreceptors / Q000473:pathology; D051379:Mice; D008822:Mice, Transgenic; D009412:Nerve Fibers / Q000378:metabolism / Q000473:pathology*; D012867:Skin / Q000294:innervation / Q000473:pathology*; D020886:Somatosensory Disorders / Q000209:etiology* / Q000473:pathology*; D013482:Superoxide Dismutase; D043222:Ubiquitin Thiolesterase / Q000378:metabolism
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase; D043222:Ubiquitin Thiolesterase; C496976:Uchl1 protein, mouse
keywords: PGP9.5; SOD1G93A mouse; amyotrophic lateral sclerosis; sensory innervation