Altered iPSC-derived neurons’ sodium channel properties in subjects with Monge’s disease

• Published in: Neuroscience Vol. 288; pp. 187 - 199
• Main Authors: Zhao, H.W., Gu, X.Q., Chailangkarn, T., Perkins, G., Callacondo, D., Appenzeller, O., Poulsen, O., Zhou, D., Muotri, A.R., Haddad, G.G.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Ltd 12.03.2015
• Subjects: Action Potentials - physiology; Adult; Altitude Sickness - physiopathology; Cell Culture Techniques; Cells, Cultured; Chronic Disease; chronic mountain sickness; Fibroblasts - cytology; Fibroblasts - physiology; Humans; Immunohistochemistry; induced pluripotent stem cells; Induced Pluripotent Stem Cells - cytology; Induced Pluripotent Stem Cells - physiology; Male; Na+ channel; Neural Stem Cells - cytology; Neural Stem Cells - physiology; Neurogenesis - physiology; Neurology; neurons; Neurons - cytology; Neurons - physiology; Patch-Clamp Techniques; Peru; Sodium Channels - metabolism; Young Adult; Peru; SUMO; CMS; Na+ channel; neurons; ES; RIPA; AP; DMEM; iPSCs; NPCs; chronic mountain sickness; TTX; induced pluripotent stem cells; EB; Dulbecco’s Modified Eagle Medium; Na + channel; Small Ubiquitin-like Modifier; action potential; embryoid body; neural progenitor cells; embryonic stem; radioimmunoprecipitation assay buffer; tetrodotoxin; Na(+) channel
• ISSN: 0306-4522; 1873-7544; 1873-7544
• DOI: 10.1016/j.neuroscience.2014.12.039

•iPSC-derived neurons are generated from CMS and non-CMS subjects.•Neurons are characterized and neuronal properties are compared.•CMS neurons are much less excitable than non-CMS neurons.•The decreased excitability in CMS neurons is due to alteration in Na+ channel properties. Monge’s disease, also...