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的肺病，介于生存极限和儿童早期，仍然是疾病和死亡的主要原因。发现治疗这些疾病的新疗法的研究受到这个年龄段的人很少接触到正常和患病的人肺的限制。罗彻斯特大学医学中心(URMC)应NHLBI RFA-HL-14-007的要求提交了本申请，该申请要求达成一项合作协议(U01)，作为肺发育计划分子图谱(LongMAP)的人体组织核心(HTC)。HTC将负责识别和管理组织源站点，以收集必要的组织，以实现该计划的总体目标，正如RFA中所述，该计划的总体目标是：通过创建一个全面的发育后期肺的分子图谱，利用可供研究社区使用的数据和试剂，建立一个开放访问的参考资源。该图谱将把基因和蛋白质表达谱、转录组、表观基因组和其他分子特征与高分辨率解剖学信息相结合，提供发育中肺的功能或解剖定义的细胞类型的分子图谱。作为HTC，URMC将收集、处理、储存正常的胎儿晚期和儿童早期人类肺样本，并将其分发到四个LUNGMAP研究中心(RCS)。由于以标准化的方式和尽可能少的缺血引起的伪影恢复肺的担忧和复杂性，以及满足所有法律和道德标准的需要，我们选择了通过器官共享联合网络的元素进行工作，该非营利性组织管理着美国联邦签约的器官采购和移植网络(OPTN)。具体地说，我们选择与两个非营利性组织合作，这些组织被转介给两个非营利性组织，即国家疾病研究交流中心(NDRI)和国际医学促进研究所(IIAM)。NDRI和IIAM致力于与OPTN和因怀孕而接近他们的家庭合作，因为怀孕预计会导致围产期死亡。我们在URMC将准备捐赠的器官以满足RCS的需求；我们至少准备获得锥束CT扫描，以评估总体积、血管和呼吸道结构，准备固定的、包埋的和切片的组织，并通过组织分离和流动激活细胞分选分离特定细胞群。一个由高素质的病理学家组成的工作组将为HTC提供咨询，开发标准的生物信息库和评估工具，并审查样本，以确保选择进行研究的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收集的数据。我们还将从一开始就制定计划，以便在主要的LUNGMAP资金之后继续保存和获得这些稀有样品。(摘要结束)

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)