ANIMAL MODELS OF HEREDITARY RETINAL DEGENERATION

遗传性视网膜变性的动物模型

• 批准号: 2161061
• 负责人: GREGORY M ACLAND
• 金额: $ 42.9万
• 依托单位: CORNELL UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 1992
• 资助国家: 美国
• 起止时间: 1992-12-01 至 1994-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/2161061
• 关键词: animal breeding; animal colony; disease /disorder model; dogs; genetic disorder; genetic strain; model design /development; mutant; retina degeneration; tissue resource /registry; vision disorders

This application proposes continuance of the project "Canine Models of Hereditary Retinal Degeneration", as a national research resource for studies of hereditary retinal degenerations. The need for these canine mutants in research studies is addressed, as is the importance of maintaining them in a centralized breeding and distribution facility. Examples of research studies that will utilize the colony, and their significance, are summarized. Specific and comprehensive details of such research are not included, however, as these form part of the peer reviewed research programs (funded by ROI or other support) of those individual investigators utilizing the colony's resources. Four specific mutant strains of dog, each transmitting a different, unique gene for a well-characterized, autosomal recessive hereditary retinal degeneration (rcd1: Rod-Cone Dysplasia Type 1; prcd: Progressive Rod-Cone Degeneration; erd: Early Retinal Degeneration; cd: Cone Degeneration) together with appropriate nonaffected control dogs, will be bred, maintained and made available for approved research investigations. The progeny of these dogs will be distributed to approved research investigators either directly or by collection, processing and distribution of requested tissues. DNA from representative individuals and from informative pedigrees of each mutant strain will be preserved and made available to all approved investigators. The primary aims of this project are to ensure continued availability of the respective mutations and to promote their utilization by research investigators. Utilization will be promoted by solicitation of requests from investigators. Responses will be reviewed for scientific merit and priorized by an independent Retinal Research Resources Committee (RRRC), appointed and chaired by appropriate NEI/NIH staff. Allocation of distributable resources (such as dogs, tissues, DNA) from the colony will be according to priorities set by the RRRC. Investigators will be assisted in the development and implementation of protocols for optimal utilization of these mutants.

该应用程序提出了项目的延续 遗传性视网膜变性”，作为国家研究资源 遗传性视网膜变性的研究。 这些犬的需求 研究研究中的突变体以及 将它们维持在集中式的繁殖和分销设施中。 将利用殖民地及其的研究示例 显着性，总结。 此类的具体和全面的细节 但是，研究不包括在内，因为这些构成了同伴的一部分 审查了这些计划的研究计划（由ROI或其他支持资助） 个人调查人员利用殖民地的资源。 狗的四个特定突变菌株，每种都会传递不同的狗 独特的基因，用于特征良好的常染色体隐性遗传 视网膜变性（RCD1：杆孔发育不良1型； PRCD：渐进式 杆锥变性； ERD：早期视网膜变性； CD：锥 变性）与适当的未影响对照犬一起，将是 育种，维护和提供可用于批准的研究调查。 这些狗的后代将分配给批准的研究 调查人员直接或通过收集，处理和 要求组织的分布。 来自代表个体的DNA 并保留每个突变株的典范 并提供给所有批准的调查员。 该项目的主要目的是确保继续可用 各自的突变并通过研究促进其利用 调查人员。 利用将通过征求请求来促进 来自调查人员。 响应将被审查以获得科学功绩和 由独立的视网膜研究资源委员会（RRRC）的首选， 由适当的NEI/NIH工作人员任命和主持。 分配可分配资源（例如狗，组织，DNA） 殖民地将根据RRRC设定的优先级。 调查人员将得到协助开发和实施 这些突变体最佳利用的方案。