Involvement of Peripheral Nerves in the Transgenic PLP-α-Syn Model of Multiple System Atrophy: Extending the Phenotype

摘要: Multiple system atrophy (MSA) is a fatal, rapidly progressive neurodegenerative disease with (oligodendro-)glial cytoplasmic α-synuclein (α-syn) inclusions (GCIs). Peripheral neuropathies have been reported in up to 40% of MSA patients, the cause remaining unclear. In transgenic mouse model featuring GCI-like inclusion pathology based on PLP-promoter driven overexpression human α-syn oligodendroglia motor and non-motor deficits are associated MSA-like neurodegeneration. Since also expressed Schwann cells we aimed investigate whether peripheral nerves anatomically functionally affected PLP-α-syn model. Results To this end, heat/cold as well mechanical sensitivity tests were performed. Furthermore, vivo ex nerve conduction G-ratios sciatic analyzed, thermosensitive ion channel mRNA expression dorsal root ganglia (DRG) was assessed. The presence subtle behavioral impairments. G-ratio nerve, velocity myelinated unmyelinated primary afferents channels sensory neurons, however, similar wildtype mice.