ANIMAL MODELS OF HEREDITARY RETINAL DEGENERATION

本申请书建议延续“犬科动物模型”项目。 遗传性视网膜变性“，作为一个全国性的研究资源 遗传性视网膜变性的研究。对这些狗的需求 以及突变体在研究研究中的重要性 将它们保存在集中的繁殖和分发设施中。 将利用该殖民地的研究研究的例子，以及他们的 意义，并对其进行总结。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) 个体调查人员利用殖民地的资源。 四种特定的狗突变品系，每种都传播一种不同的， 一种特征良好的常染色体隐性遗传病的独特基因 视网膜变性(RCD1：视杆-视锥发育不良1型；PRCD：进行性 视杆-视锥细胞变性；ERD：早期视网膜变性；CD：视锥细胞 退化)与适当的不受影响的对照犬一起，将被 繁殖、维护并提供给经批准的研究调查使用。 这些狗的后代将被分发给经批准的研究 调查人员可以直接或通过收集、处理和 所需组织的分布。有代表性的个体的DNA 来自每个突变菌株的信息来源的谱系将被保存 并提供给所有经批准的调查人员。 该项目的主要目标是确保持续提供 各自的突变并通过研究促进其利用 调查人员。将通过征集请求来促进利用率 来自调查人员。将审查答复的科学价值，并 由一个独立的视网膜研究资源委员会(RRRC)优先考虑， 由NEI/NIH适当的工作人员任命和担任主席。 可分配资源(如狗、组织、DNA)的分配 殖民地将根据RRRC设定的优先顺序进行。 将协助调查人员制定和实施 最佳利用这些突变体的方案。

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.