ANIMAL MODELS OF HEREDITARY RETINAL DEGENERATION

本申请建议继续项目“犬模型的 遗传性视网膜变性”，作为国家研究资源， 遗传性视网膜退化的研究。 对这些犬类的需求 突变体在研究中的重要性， 将它们集中饲养和分配。 将利用殖民地的研究实例及其 的意义，进行了总结。 具体和全面的细节， 然而，研究不包括在内，因为这些研究是同行的一部分。 审查研究计划（由ROI或其他支持资助）， 利用殖民地资源的独立调查员。 四种变异的狗，每一种都传播不同的， 一个独特的基因，为一个良好的特点，常染色体隐性遗传 视网膜变性（rcd 1：视杆-视锥发育不良1型; prcd：进行性 视杆-视锥变性; erd：早期视网膜变性; cd：视锥 退化）以及适当的未受影响的对照犬， 饲养、维护并提供给批准的研究调查。 这些狗的后代将被分配到批准的研究 调查人员直接或通过收集、处理和 分发所需的组织。 代表性个体的DNA 并从每个突变株的信息谱系将被保留 并提供给所有批准的研究者。 该项目的主要目的是确保持续提供 各自的突变，并通过研究促进其利用 investigators. 将通过征求请求来促进利用 从调查员。 将根据科学价值审查答复， 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。 分配可分配资源（如犬、组织、DNA）， 殖民地将根据RRRC设定的优先级。 将协助研究者制定和实施 这些突变体的最佳利用方案。

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.