Improvement of impaired electrical activity in NPC1 mutant cortical neurons upon DHPG stimulation detected by micro-electrode array.

Brain Res

Albrecht-Kossel-Institute for Neuroregeneration, Rostock University Medical Center, Gehlsheimer Straße 20, 18147 Rostock, Germany; Centre for Transdisciplinary Neuroscience Rostock, Rostock University Medical Center, Gehlsheimer Straße 20, 18147 Rostock, Germany. Electronic address:

Published: September 2018

Niemann-Pick Type C1 (NPC1) disease is an autosomal recessive neurodegenerative disease characterized by an excessive accumulation of unesterified cholesterol in late endosomes/lysosomes. Patients with NPC1 disease show a series of symptoms in neuropathology, including a gradually increased loss of motor control and seizures. However, mechanism of the neurological manifestations in NPC1 disease is not fully understood yet. In this study, we utilized the micro-electrode array (MEA) to analyze the spontaneous extracellular electrical activity in cultivated cortical neurons of the NPC1 mutant (NPC1) mouse. Our results show a decrease of the spontaneous electrical activity in NPC1 neuronal network when compared to wild type neurons, as indicated by the decreased spike rate, burst rate, event rate, and the increased burst period and event period. Application of 3,5-dihydroxyphenylglycine (DHPG), a specific agonist of group I metabotropic glutamate receptors, improved the electrical activity of the NPC1 neuronal network, suggesting that DHPG can be used as a potential therapeutic strategy for recovery of the electrical activity in NPC1 disease.

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http://dx.doi.org/10.1016/j.brainres.2018.05.009DOI Listing

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