ANIMAL MODEL OF TRANSMISSIBLE NEUROFIBROMAS

描述：拟议的研究计划将调查 外周神经外胚层细胞类型的肿瘤性转化 神经系统。一种独特的动物系统，丹自鱼型神经纤维瘤病(DNF)， 将被用来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是由一种可传染的， 亚细胞因子，似乎是唯一一种可传播的 肿瘤涉及任何神经外胚层来源的细胞类型。这项研究将 强调糖尿病神经营养不良的病原学特征及其应用 药物作为一种工具从三个方面了解该病的发病机制 密切相关的目标：(1)假设将被检验为一个新的群体 在DNF肿瘤中发现的染色体外DNA是DNA的病原体， (2)这种称为DNFX的试剂将被表征以确定序列 DNA基因组和RN转录本的关系以及DNFX与 确定DNA基因组和RNA转录本的序列，并 检查DNFX与已定义的病毒家族和 类似病毒的毒剂。(3)DNFX编码的蛋白质将被鉴定并 为确定DNFX蛋白在体内的分布而开发的抗体 并鉴定可能与DNFX蛋白结合的细胞蛋白。

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.