Back to Search
Start Over
Improvement of impaired electrical activity in NPC1 mutant cortical neurons upon DHPG stimulation detected by micro-electrode array.
- Source :
-
Brain Research . Sep2018, Vol. 1694, p87-93. 7p. - Publication Year :
- 2018
-
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. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 00068993
- Volume :
- 1694
- Database :
- Academic Search Index
- Journal :
- Brain Research
- Publication Type :
- Academic Journal
- Accession number :
- 130357485
- Full Text :
- https://doi.org/10.1016/j.brainres.2018.05.009