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Involvement of sensory innervation in the skin of SOD1 G93A ALS mice
Author(s) -
Rubio Miguel A.,
HerrandoGrabulosa Mireia,
Vilches Jorge J.,
Navarro Xavier
Publication year - 2016
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1111/jns.12164
Subject(s) - amyotrophic lateral sclerosis , sod1 , neurodegeneration , immunostaining , pathology , sensory system , axonal degeneration , medicine , sensory nerve , neuroscience , anatomy , immunohistochemistry , biology , disease
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.