Pulmonary Hypertension Breakthrough Initiative

肺动脉高压突破计划

• 批准号: 8756641
• 负责人: Serpil C. Erzurum
• 金额: $ 255.8万
• 依托单位: UNIVERSITY OF COLORADO DENVER
• 依托单位国家: 美国
• 财政年份: 2014
• 资助国家: 美国
• 起止时间: 2014-09-15 至 2018-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8756641
• 关键词: Abbreviations; Alabama; American; Bioinformatics; Blood; Blood Pressure; Blood Vessels; Blood specimen; Cardiac; Cardiovascular system; Cataloging; Catalogs; Categories; Cell Line; Cell Separation; Cells; Clinic; Clinical; Clinical Data; Colorado; Consult; Consultations; DNA analysis; Data; Data Analyses; Data Coordinating Center; Development; Diagnosis; Diagnostic; Diagnostic tests; Disease; Education; Enrollment; Funding; Future; Gene Expression; Genetic; Genomics; Goals; Heart; Human; Incidence; Informatics; Inherited; Institutes; Institutional Review Boards; Investigation; Lung; Lung Transplantation; Lung diseases; Medical Education; Medical Research; Michigan; Monoclonal Antibody R24; National Heart, Lung, and Blood Institute; Pathologic; Pathology; Patients; Pennsylvania; Pharmaceutical Preparations; Phenotype; Physiological; Predisposition; Process; Pulmonary Hypertension; Pulmonary artery structure; RNA; Recording of previous events; Research; Research Design; Research Infrastructure; Research Personnel; Research Support; Resources; Risk Factors; Role; Site; Specimen; Time; Tissues; Translations; Transplant Recipients; Transplantation; Universities; Woman; Work; World Health Organization; abstracting; base; biobank; blood pump; clinical care; database of Genotypes and Phenotypes; effective therapy; electronic data; flexibility; genome wide association study; heart cell; high throughput analysis; human tissue; hypertension control; material transfer agreement; meetings; member; mortality; next generation sequencing; novel; novel diagnostics; public health relevance; pulmonary arterial hypertension; success

DESCRIPTION (provided by applicant): Pulmonary Arterial Hypertension (PAH) is a heterogeneous group of diseases with high mortality, difficult diagnosis, limited available treatment, and no cure. The Pulmonary Hypertension Breakthrough Initiative (PHBI) is dedicated to comprehensive biobanking of human lungs with PAH and controls, supported by a detailed clinical annotation of the accrued specimens. Having started in 2006, the PHBI successfully developed a novel and unique infrastructure, whose success relied on the active participation of a highly integrated network of university-based sites with extensive expertise in each of the spheres of competency: excellence in clinical care of PAH (including patient accruals), lung transplantation, pathology, genetics, genomics, and cell isolation. The goal of this application is to leverage these unparalled resources and unique expertise into an R24-supported initiative to direct the bank to accrue specimens of disease-specific groups, which will be highly integrated with pathologic, genetic, and genomic subphenotypes pertaining to not only lung and blood specimens, but now expanding to the failing PAH heart. This proposal will be highly synergistic with the presently supported initiatives by the NHLBI in the field. The flexibilty of our infrastructure and governance will allow us to be decidedly responsive and integrated with any future NHLBI initiatives. We propose that this unique infrastructure will serve the pressing roles of advancing the translation of key discoveries to the patients with PAH and to aid in new discoveries that will have a long lasting impact in the field. The following specific aims will be pursued: Specific Aims of the Proposal 1) To establish a comprehensive biorepository of the specimens from subjects with pulmonary arterial hypertension and failed donor controls. 2) To provide the infrastructure to support human tissue based research in PAH via integrated cores devoted to Administration, Tissue Handling and Pathology, Cell Line Development, and Genomic Characterization and Cataloging. 3) To develop and implement an interface for consultation of research design and analysis to enhance broader scientific investigation.

描述（由申请人提供）：肺动脉高压（PAH）是一组异质的疾病，具有高死亡率，困难诊断，有限的治疗方法，无法治愈。肺动脉高压突破倡议（PHBI）致力于对人类肺部使用PAH和对照组的全面生物群，并得到了应计标本的详细临床注释的支持。 PHBI成立于2006年，成功地开发了一种新颖而独特的基础设施，其成功依赖于高度集成的基于大学网站的网络的积极参与，这些网站在每个能力领域具有广泛的专业知识：PAH临床护理卓越（包括患者应计）（包括患者应计），肺移植，病理，病理，病理，病理，遗传学，遗传学，属性，属性，属性，属性，属性，属性，属性，属性。该应用程序的目的是利用这些无与伦比的资源和独特的专业知识，成为R24支持的计划，以指导银行累积特定疾病特异性群体的标本，这些群体将与病理学，遗传和基因组基因组亚型高度融合，这些型不仅与肺部和血液标本相关，而且还扩展到了失败的Pah Heart。该提案将与NHLBI目前在该领域的支持计划相协同。我们的基础设施和治理的灵活性将使我们能够完全响应并与任何未来的NHLBI计划融合在一起。我们建议，这种独特的基础设施将为PAH患者的转化转化为将主要发现转换的紧迫作用，并帮助他们在该领域产生持久影响的新发现。将追求以下具体目标： 提案的具体目的 1）从患有肺动脉高压和供体控制失败的受试者中建立标本的综合生物座。 2）提供基础设施，通过专门用于给药，组织处理和病理学，细胞系发育以及基因组表征和分类的综合核心来支持PAH中基于人体组织的研究。 3）开发和实施界面以咨询研究设计和分析，以增强更广泛的科学研究。