Biorepository for Investigation of Neonatal Diseases of Lung-Normal (BRINDL-NL)

描述（由申请人提供）：起源于肺泡形成时期（生存能力极限和幼儿期之间）的肺部疾病仍然是疾病和死亡的主要原因。发现这些疾病的新疗法的研究受到该年龄组中正常和患病人类肺部的罕见性的限制。罗切斯特大学医学中心（URMC）提交本申请，以回应NHLBI RFA-HL-14-007，该申请要求提交一份合作协议（U 01），作为肺发育计划分子图谱（LungMAP）的人体组织核心（HTC）。HTC将负责识别和管理组织来源部位，以收集必要的组织，以满足本计划的总体目标，如RFA中所述，“通过创建晚期发育肺的综合分子图谱，为研究界提供数据和试剂，建立开放获取的参考资源。该图谱将整合基因和蛋白质表达谱、转录组、表观基因组和其他具有高分辨率解剖信息的分子表征，以提供发育中的肺中功能或解剖学定义的细胞类型的分子谱。“作为HTC，URMC将收集、处理、存款和分发正常晚期胎儿和早期儿童人类肺样本给四个肺MAP研究中心（RC）。由于以标准化的方式恢复肺部的关注和复杂性，以及尽可能少的缺血引起的伪影，以及满足所有法律的和道德标准的需要，我们选择了通过器官共享联合网络的元素来工作，该非营利组织管理国家的联邦签约器官采购和移植网络（OPTN）。具体来说，我们选择了与两个非营利组织合作，他们被推荐了适合研究的非移植捐赠器官，国家疾病研究交流中心（NDRI）和国际医学促进研究所（IIAM）。国家减贫研究所和国际妇女研究所致力于与被占领土和因怀孕可能导致围产期死亡而向其求助的家庭合作。我们将在URMC准备捐赠器官以满足RC的需求;至少我们准备获得锥形束CT扫描以评估总体积、血管和气道结构，准备固定、包埋和切片组织，并通过组织解离和流动激活细胞分选分离特定细胞群。一个由高素质病理学家组成的工作组将为HTC提供咨询，以开发标准生物储存库和评估工具，并审查标本，以确保所选研究组织的质量和正常性。我们将与DCC密切合作，以确保兼容的数据系统和最终开放访问所收集的数据。我们还将从一开始就制定计划，以便在获得LungMAP主要资助后继续保存和提供这些稀有样本。(End摘要）

DESCRIPTION (provided by applicant): Lung disease originating in the period of alveologenesis, between the limit of viability and early childhood, remains a major cause of illness and death. Research to discover novel treatments for these disorders is limited by the rarity of access to normal and diseased human lung in this age group. The University of Rochester Medical Center (URMC) submits this application in response to NHLBI RFA-HL-14-007 requesting proposals to enter into a cooperative agreement (U01) to serve as the Human Tissue Core (HTC) for the Molecular Atlas of Lung Development Program (LungMAP). It will be the responsibility of the HTC to identify and manage tissue source sites to collect the necessary tissues to meet the overall goal of this program which is, as stated in the RFA, "to build an open-access reference resource by creating a comprehensive molecular atlas of the late-stage developing lung with data and reagents available to the research community. The atlas will integrate gene and protein expression profiles, transcriptome, epigenome, and other molecular characterizations with high-resolution anatomical information to provide molecular profiles of functionally or anatomically defined cell types in the developing lung." As the HTC, URMC will collect, process, deposit, and distribute normal late fetal and early childhood human lung samples to four LungMAP Research Centers (RCs). Because of the concern and complexity in recovering the lungs in a standardized manner and with as little ischemia-induced artifact as possible, and the need to meet all legal and ethical standards, we have chosen to work through elements of the United Network of Organ Sharing, the non-profit organization that manages the nation's federally contracted Organ Procurement and Transplantation Network (OPTN). Specifically, we have chosen to work with two non-profit organizations to whom are referred non-transplantable donated organs suitable for research, The National Disease Research Interchange (NDRI) and the International Institute for the Advancement of Medicine (IIAM). NDRI and IIAM are committed to working with the OPTN and with families who approach them because of a pregnancy that is expected to result in a perinatal death. We at URMC will prepare the donated organs to meet the needs of the RCs; at minimum we are prepared to obtain cone beam CT scans for evaluation of total volume, vascular and airway structure, prepare fixed, embedded and sectioned tissue and isolate specific cell populations by tissue dissociation and flow activated cell sorting. A working group of highly qualified Pathologists will consult for the HTC, to develop standard biorepository and assessment tools and to review specimens to assure quality and normality of the tissues chosen for study. We will work closely with the DCC to assure compatible data systems and ultimate open access to the collected data. We will also, from the inception, develop plans for the continued preservation and availability of these rare samples following the primary LungMAP funding. (End of Abstract)