ANIMAL MODEL OF TRANSMISSIBLE NEUROFIBROMAS

传染性神经纤维瘤的动物模型

• 批准号: 2692726
• 负责人: MICHAEL C SCHMALE
• 金额: $ 19.08万
• 依托单位: UNIVERSITY OF MIAMI CORAL GABLES
• 依托单位国家: 美国
• 财政年份: 1998
• 资助国家: 美国
• 起止时间: 1998-07-08 至 2001-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2692726
• 关键词: ANIMAL; MODEL; TRANSMISSIBLE; NEUROFIBROMAS

DESCRIPTION: The proposed research program will investigate mechanisms of neoplastic transformation of neuroectodermal cell types in the peripheral nervous system. A unique animal system, damselfish neurofibromatosis (DNF), will be used to address basic questions in the pathogenesis of tumors such as neurofibromas and neurofibrosarcomas. DNF is caused by a transmissible, sub-cellular agent and appears to be the only example of a transmissible tumor involving any neuroectodermally derived cell type. This research will emphasiz characterization of the etiologic agent of DNF and the use of this agent as a tool to understand pathogenesis in this disease through three closely related aims: (1) The hypothesis will be tested that a novel group of extrachromosomal DNAs found in DNF tumors is the etiologic agent of DNA, (2) This agent, termed DNFX, will be characterized to determine the sequence of the DNA genome and RN transcripts and to examine the relationship of DNFX to determine the sequence of the DNA genome and RNA transcripts and to examine the relationship of DNFX to defined families of viruses and virus-like agents. (3) Proteins encoded by DNFX will be identified and antibodies developed to determine the distribution of DNFX proteins in tissues and to identify cellular proteins that may be boun by DNFX proteins.

描述：拟议的研究计划将调查的机制， 外周神经外胚层细胞类型的肿瘤转化 神经系统 一个独特的动物系统，雀鲷神经纤维瘤病（DNF）， 将用于解决肿瘤发病机制中的基本问题， 神经纤维瘤和神经纤维肉瘤。 DNF是由一种可传播的， 亚细胞因子，似乎是唯一的例子， 涉及任何神经外胚层衍生细胞类型的肿瘤。 这项研究将 强调DNF病原体的特征及其应用 作为一种工具，通过三种方法来了解这种疾病的发病机制 密切相关的目的：（1）假设将被测试，一个新的群体， 在DNF肿瘤中发现的染色体外DNA是DNA的病原体， (2)这种被称为DNFX的试剂将被表征以确定序列 的DNA基因组和RN转录本，并检查DNFX的关系， 确定DNA基因组和RNA转录物的序列， 检查DNFX与定义的病毒家族的关系， 病毒样物质 (3)将鉴定DNFX编码的蛋白质， 开发抗体以确定DNFX蛋白在 组织和鉴定可能由DNFX蛋白质形成的细胞蛋白质。