细胞间通讯的功能和调节-猫眼课题宝

细胞间通讯的功能和调节

批准号：

7314798

负责人：

DAVID L PAUL

金额：

$42.25万

依托单位：

HARVARD MEDICAL SCHOOL

依托单位国家：

美国

项目类别：

财政年份：

1986

资助国家：

美国

项目状态：

已结题

起止时间：

1986-12-01 至 2011-06-30

来源：

https://reporter.nih.gov/project-details/7314798

关键词：

Ablation Action Potentials Address Affect Animal Model Animals Astrocytes Auditory Brainstem Responses Axon Behavior Behavior Control Biolistics Biological Assay Charcot-Marie-Tooth Disease Complex Connexin 43 Connexins Coupling Cytoplasm Development Disease Dyes Electric Stimulation Employee Strikes Exhibits Extracellular Space Fiber Gap Junctions Gene Targeting Gene Transfer Techniques Green Fluorescent Proteins Hela Cells High-Frequency Hearing Loss Homeostasis In Vitro Injection of therapeutic agent Knock-out Knockout Mice Life Localized Location Maintenance Mediating Membrane Methodology Microelectrodes Microinjections Modeling Molecular Movement Mutation Myelin Myelin Sheath Neuraxis Neuroglia Neurons Neuropathy Nodal Numbers Oligodendroglia Pathway interactions Pelizaeus-Merzbacher Disease Peripheral Nervous System Phenotype Potassium Channel Preparation Procedures Property Proteins Ranvier&apos s Nodes Regulation Relative (related person)Reporting Role Schwann Cells Slice Spinal Cord Structure System Testing Transgenes Transgenic Animals Transgenic Organisms base cell type central nervous system demyelinating disorder cost desire disease-causing mutation gene gun human GJB2 protein in vivo intercellular communication mutant myelination neuronal cell body research study sciatic nerve spiral ganglion tissue/cell culture uptake

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中文摘要

翻译

描述（由申请人提供）：以前，我们发现连接蛋白32（Cx32）的突变导致一种形式的腓骨肌萎缩症，一种周围神经系统（PNS）的脱髓鞘神经病。此外，我们在小鼠敲除研究中显示，Cx32和Cx47提供了中枢神经系统（CNS）正常髓鞘形成所必需的冗余功能，最近发现Cx47突变导致Pelizaeus-Merzbacher样疾病（PMLD），其特征在于CNS髓鞘异常，证实了这一观察结果。因此，CNS和PNS中髓鞘的维持需要连接蛋白表达。然而，目前还不清楚少突胶质细胞和许旺细胞如何使用连接蛋白以及为什么它们对正常髓鞘形成至关重要。我们建议使用靶向基因消融和连接蛋白通道活性的功能分析相结合来定义连接蛋白在髓鞘形成中的单独和相互作用的作用以及突变导致疾病的原因。'.

英文摘要

DESCRIPTION (provided by applicant): Previously, we showed that mutations in connexin32 (Cx32) cause a form of Charcot-Marie-Tooth disease, a demyelinating neuropathy of the peripheral nervous system (PNS). In addition, we showed in mouse knockout studies Cx32 and Cx47 provide redundant functions necessary for normal myelination in the central nervous system (CNS), an observation confirmed by the recent discovery that Cx47 mutations cause Pelizaeus-Merzbacher-Like disease (PMLD), characterized by abnormalities in CNS myelin. Thus, maintenance of myelin in both CNS and PNS requires connexin expression. However, it is not at all clear how oligodendrocytes and Schwann cells use connexins and why they are critical for normal myelination. We propose to define the separate and interacting roles of connexins in myelination and why mutations cause disease using a combination of targeted gene ablation and functional analysis of connexin channel activity. '.

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