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Changes in Ionic Co...
Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype
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- Namer, Barbara (författare)
- Univ Erlangen Nurnberg, Dept Physiol & Pathophysiol, Erlangen, Germany.;Heidelberg Univ, Dept Anesthesiol, Mannheim, Germany.
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- Ørstavik, Kirstin (författare)
- Oslo Univ Hosp, Sect Clin Neurophysiol, Dept Neurol, Rikshosp, Oslo, Norway.
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- Schmidt, Roland (författare)
- Uppsala universitet,Klinisk neurofysiologi
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- Mair, Norbert (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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- Kleggetveit, Inge Petter (författare)
- Oslo Univ Hosp, Sect Clin Neurophysiol, Dept Neurol, Rikshosp, Oslo, Norway.
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- Zeidler, Maximillian (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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- Martha, Theresa (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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- Jorum, Ellen (författare)
- Heidelberg Univ, Dept Anesthesiol, Mannheim, Germany.;Univ Oslo, Inst Clin Med, Fac Med, Oslo, Norway.
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- Schmelz, Martin (författare)
- Heidelberg Univ, Dept Anesthesiol, Mannheim, Germany.
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- Kalpachidou, Theodora (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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- Kress, Michaela (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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- Langeslag, Michiel (författare)
- Med Univ Innsbruck, Div Physiol, Dept Physiol & Med Phys, Innsbruck, Austria.
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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. (creator_code:org_t)
- 2017-07-14
- 2017
- Engelska.
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Ingår i: Frontiers in Neurology. - : FRONTIERS MEDIA SA. - 1664-2295. ; 8
- Relaterad länk:
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https://doi.org/10.3...
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https://uu.diva-port... (primary) (Raw object)
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https://www.frontier...
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https://urn.kb.se/re...
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https://doi.org/10.3...
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Abstract
Ämnesord
Stäng
- 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.
Nyckelord
- Fabry disease
- lysosomal storage disorder
- single fiber recordings
- neuronal excitability
- electrophysiology
- nociception
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Namer, Barbara
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Ørstavik, Kirsti ...
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Schmidt, Roland
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Mair, Norbert
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Kleggetveit, Ing ...
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Zeidler, Maximil ...
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visa fler...
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Martha, Theresa
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Jorum, Ellen
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Schmelz, Martin
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Kalpachidou, The ...
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Kress, Michaela
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Langeslag, Michi ...
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visa färre...
- Artiklar i publikationen
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Frontiers in Neu ...
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Uppsala universitet