کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8839716 | 1613750 | 2018 | 7 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Improvement of impaired electrical activity in NPC1 mutant cortical neurons upon DHPG stimulation detected by micro-electrode array
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
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چکیده انگلیسی
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 (NPC1â/â) mouse. Our results show a decrease of the spontaneous electrical activity in NPC1â/â 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 NPC1â/â neuronal network, suggesting that DHPG can be used as a potential therapeutic strategy for recovery of the electrical activity in NPC1 disease.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain Research - Volume 1694, 1 September 2018, Pages 87-93
Journal: Brain Research - Volume 1694, 1 September 2018, Pages 87-93
نویسندگان
Xiao Feng, Benjamin M. Bader, Fan Yang, Monica Segura, Luise Schultz, Olaf H.-U. Schröder, Arndt Rolfs, Jiankai Luo,