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. 2017 Jul 14;8:335. doi: 10.3389/fneur.2017.00335

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Copyright © 2017 Namer, Ørstavik, Schmidt, Mair, Kleggetveit, Zeidler, Martha, Jorum, Schmelz, Kalpachidou, Kress and Langeslag.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Electrical properties of Gla^−/0 cultured sensory neurons and activity-dependent slowing of human C-fibers. (A) Action potential (AP) generation upon prolonged depolarization significantly changes in adult Gla^−/0 nociceptors. In matured nociceptors, the constant firing rate in Gla^−/0 nociceptive neurons is decreased (left panel) and the facilitative and adaptive firing frequency is increased (middle and right panels, respectively). (B) Furthermore, nociceptors from Gla^−/0 mice show an elevated amount of neurons that display a facilitative firing at adult ages and a reduction in the neurons that generate a constant AP firing frequency. (C) Activity-dependent conduction velocity slowing (ADS) in C-fibers from patients with M. Fabry and healthy elderly controls showed no significant differences in ADS between heat-responsive and -unresponsive C-fibers. In the left panel, ADS of mechano-responsive C-fibers with (CMH) and without heat response (CM) to electrical stimulation with different frequencies (0.125, 0.25, and 0.5 Hz) and the sum of these ADS steps are depicted. (D) ADS of sympathetic C-fibers is shown. No significant differences were found in ADS between patients with M. Fabry and healthy controls.