| First Author | Namer B | Year | 2017 |
| Journal | Front Neurol | Volume | 8 |
| Pages | 335 | PubMed ID | 28769867 |
| Mgi Jnum | J:311223 | Mgi Id | MGI:6766304 |
| Doi | 10.3389/fneur.2017.00335 | Citation | Namer B, et al. (2017) Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype. Front Neurol 8:335 |
| abstractText | 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 alpha-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 Ca(2+) currents was decreased in Gla(-/0) 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. |
