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Title: DEFECTS OF SYNAPTIC VESICLE TURNOVER AT EXCITATORY AND INHIBITORY SYNAPSES IN NIEMANN-PICK C1-DEFICIENT NEURONS
Author: Xu, S. ; Zhou, S. ; Xia, D. ; Xia, J. ; Chen, G. ; Duan, S. ; Luo, J.
Source: NEUROSCIENCE
Issued Date: 2010
Volume: 167, Issue:3, Pages:608-620
Keyword: cholesterol ; synaptic vesicle recycling ; readily releasable pool ; spine ; READILY RELEASABLE POOL ; CULTURED HIPPOCAMPAL-NEURONS ; C-DISEASE ; NERVE-TERMINALS ; DISTAL AXONS ; CELL-BODIES ; CHOLESTEROL ; PROTEIN ; PLASTICITY ; NEURODEGENERATION
Subject: Neurosciences & Neurology
Corresponding Author: Luo, J (reprint author), Zhejiang Univ, Sch Med, Inst Neurosci, Dept Neurobiol, 388 Yu Hangtang St, Hangzhou 310058, Zhejiang, Peoples R China,luojianhong@zju.edu.cn
English Abstract: Niemann-Pick disease type C (NPC) is a progressive neurodegenerative disorder characterized by accumulation of free cholesterol in late endosomes/lysosomes. The pathological basis for the disease is poorly understood. In the present study, electrophysiological and fluorescent dye studies were applied to examine neuron-specific functions of Niemann-Pick disease type Cl (NPC1) and to determine whether excitatory and inhibitory synapses are differentially impaired by NPC1 deficiency. Densities of spines and postsynaptic receptor clusters were not affected by NPC1 deficiency over the period examined. However, drastic defects on exocytosis were found both in glutamatergic and GABAergic synapses. The defects were caused in part by a delay in the time required for replacement of excytosed vesicles with new fusion-competent ones. Moreover, we found that the delay of synaptic vesicle turnover was longer in inhibitory synapses (>3 s) than in excitatory synapses (<0.2 s). These defects may be early indicators, and could provide a potential explanation for key features of the disease, such as dystonia and seizures. (C) 2010 IBRO. Published by Elsevier Ltd. All rights reserved.
Indexed Type: sci
Language: 英语
Content Type: 期刊论文
URI: http://ir.sibs.ac.cn/handle/331001/1592
Appears in Collections:神经所(总)_期刊论文

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Recommended Citation:
Xu, S.; Zhou, S.; Xia, D.; Xia, J.; Chen, G.; Duan, S.; Luo, J..DEFECTS OF SYNAPTIC VESICLE TURNOVER AT EXCITATORY AND INHIBITORY SYNAPSES IN NIEMANN-PICK C1-DEFICIENT NEURONS,NEUROSCIENCE,2010,167(3):608-620
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