UHI COBRE: PATHOGENETICS OF RECESSIVE PSEUDOXANTHOMA ELASTICUM

UHI COBRE：隐性弹性假黄瘤的发病机制

• 批准号: 7609910
• 负责人: OLIVIER LE SAUX
• 金额: $ 25.78万
• 依托单位: UNIVERSITY OF HAWAII AT MANOA
• 依托单位国家: 美国
• 财政年份: 2007
• 资助国家: 美国
• 起止时间: 2007-06-01 至 2008-05-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7609910
• 关键词: Arthritis; Blood Vessels; Computer Retrieval of Information on Scientific Projects Database; Data; Dermal; Development; Disease; Funding; Goals; Grant; Institutes; Institution; Musculoskeletal; Phenotype; Pseudoxanthoma Elasticum; Research; Research Personnel; Resources; Source; Thalassemia; United States National Institutes of Health; Work; beta Thalassemia; mouse model; research study; skin disorder

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The overall goal of this proposal is to define the pathogenetics of a rare heritable disorder with ocular, dermal and vascular phenotypes called Pseudoxanthoma elasticum or PXE. Progress in Year 4 included the continued work on new specific aims developed in the course of year 2 and exciting new results from an exploratory project intended to characterize the phenotype of a mouse model of thalassemia with respect to the PXE phenotype. The data obtained with both this exploratory poject and specific aim 2 thus far suggested that priority be given to the exploratory project. A CAMSSA (S11) submitted in the course of year 3 was reviewed and resubmitted to the National Institute of Arthritis and Musculoskeletal and Skin Diseases for a March 1, 2005 deadline. This revision capitalized on data deriving from the exploratory project related to PXE and beta-thalassemia. Anticipated goals for year 5 will include experiments under new specific aims 1 and the continued development of the exploratory project related to PXE and beta-thalassemia.

该副本是利用众多研究子项目之一 由NIH/NCRR资助的中心赠款提供的资源。子弹和 调查员（PI）可能已经从其他NIH来源获得了主要资金， 因此可以在其他清晰的条目中代表。列出的机构是 对于中心，这不一定是调查员的机构。 该提案的总体目标是用眼，皮肤和血管表型定义一种罕见的可遗传性疾病的病原体，称为pseudoxanthoma弹性或PXE。 第4年的进步包括在第二年开发的新特定目标上的继续工作，以及一个探索性项目的令人兴奋的新结果，旨在表征与PXE表型相对于Thalassia的小鼠模型的表型。到目前为止，使用此探索性督和特定目的2获得的数据表明，将优先考虑探索性项目。 在第3年的CAMSSA（S11）进行了审查，并将其重新提交给美国国家关节炎和肌肉骨骼和皮肤病研究所，以进行2005年3月1日的截止日期。这项修订资本利用了与PXE和Beta-Thalassia相关的探索性项目得出的数据。 第五年的预期目标将包括新特定目标1的实验以及与PXE和Beta-Thalassya相关的探索性项目的持续开发。