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.

本申请建议继续开展“犬类模型”项目 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。 对这些犬类的需求 解决了研究中的突变体，以及 将它们保存在集中饲养和分配设施中。 将利用殖民地的研究示例及其 的意义，进行了总结。 此类具体和全面的细节 然而，研究不包括在内，因为这些是同行的一部分 审查了这些项目的研究计划（由 ROI 或其他支持资助） 个别调查员利用殖民地的资源。 四种特定的狗突变株，每种都传播不同的、 具有明确特征的常染色体隐性遗传的独特基因 视网膜变性（rcd1：1 型视杆细胞发育不良；prcd：进行性 杆锥变性； erd：早期视网膜变性；光盘：锥体 退化）与适当的未受影响的对照狗一起，将 培育、维护并可供批准的研究调查使用。 这些狗的后代将被分配给批准的研究 调查人员直接或通过收集、处理和 分发所要求的组织。 来自代表性个体的 DNA 每个突变株的信息丰富的谱系将被保留 并提供给所有经批准的研究人员。 该项目的主要目标是确保持续可用 各自的突变并通过研究促进其利用 调查人员。 将通过征求请求来促进利用 来自调查人员。 将对答复的科学价值进行审查 由独立的视网膜研究资源委员会 (RRRC) 优先考虑， 由适当的 NEI/NIH 工作人员任命和主持。 分配可分配的资源（如狗、组织、DNA） 该殖民地将根据 RRRC 设定的优先级进行。 将协助调查人员制定和实施 最佳利用这些突变体的方案。