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

AI Article Synopsis

  • Fabry patients often first experience neuropathic pain, which is linked to changes in small nerve fibers, eventually leading to reduced sensory perception.* -
  • A study comparing Fabry patients to a Gla mouse model showed similar patterns of skin innervation and sensory abnormalities in both, indicating a shared underlying issue.* -
  • Electrophysiological tests revealed that Gla nociceptors had decreased voltage-gated Na and Ca currents, altering their function and contributing to sensory perception loss.*

Article 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). The skin innervation of Gla mice resembles that of the human Fabry patients. In Fabry diseased humans and Gla mice, we observed similar sensory abnormalities, which were also observed in nerve fiber recordings in both patients and mice. Electrophysiological recordings of cultured Gla nociceptors revealed that the conductance of voltage-gated Na and Ca currents was decreased in Gla nociceptors, whereas the activation of voltage-gated K currents was at more depolarized potentials. Conclusively, we have observed that reduced sensory perception due to small-fiber degeneration coincides with altered electrophysiological properties of sensory neurons.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5510289PMC
http://dx.doi.org/10.3389/fneur.2017.00335DOI Listing

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