Improvement of impaired electrical activity in NPC1 mutant cortical neurons upon DHPG stimulation detected by micro-electrode array.
Brain Res
; 1694: 87-93, 2018 09 01.
Article
em En
| MEDLINE
| ID: mdl-29753706
ABSTRACT
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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Base de dados:
MEDLINE
Assunto principal:
Proteínas
/
Metoxi-Hidroxifenilglicol
/
Neurônios
Limite:
Animals
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article