Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype.

ABSTRACT: The first symptom arising in many Fabry patients is neuropathic pain due to changes in small myelinated and unmyelinated fibers in the periphery, which is subsequently followed by a loss of sensory perception. Here we studied changes in the peripheral nervous system of Fabry patients and a Fabry mouse model induced by deletion of α-galactosidase A (Gla^-/0). The skin innervation of Gla^-/0 mice resembles that of the human Fabry patients. In Fabry diseased humans and Gla^-/0 mice, we observed similar sensory abnormalities, which were also observed in nerve fiber recordings in both patients and mice. Electrophysiological recordings of cultured Gla^-/0 nociceptors revealed that the conductance of voltage-gated Na⁺ and Ca²⁺ currents

SUBMITTER: Namer B 

PROVIDER: S-EPMC5510289 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature