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Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype
Univ Erlangen Nurnberg, Dept Physiol & Pathophysiol, Erlangen, Germany.;Heidelberg Univ, Dept Anesthesiol, Mannheim, Germany..
Oslo Univ Hosp, Sect Clin Neurophysiol, Dept Neurol, Rikshosp, Oslo, Norway..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Neuroscience, Clinical Neurophysiology.
Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria..
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2017 (English)In: Frontiers in Neurology, ISSN 1664-2295, E-ISSN 1664-2295, Vol. 8, article id 335Article in journal (Refereed) Published
Abstract [en]

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 a-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 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.

Place, publisher, year, edition, pages
FRONTIERS MEDIA SA , 2017. Vol. 8, article id 335
Keyword [en]
Fabry disease, lysosomal storage disorder, single fiber recordings, neuronal excitability, electrophysiology, nociception
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-331239DOI: 10.3389/fneur.2017.00335ISI: 000405462800001PubMedID: 28769867OAI: oai:DiVA.org:uu-331239DiVA, id: diva2:1149481
Available from: 2017-10-16 Created: 2017-10-16 Last updated: 2017-11-29Bibliographically approved

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