REGULATION OF LUNG DEVELOPMENT AND DISEASE

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 我们克隆了NPAS3，作为果蝇基因的小鼠同源物，tracaless，它的零突变体完全缺乏呼吸系统。我们的NPAS3基因缺失的小鼠中有90%在出生后几个小时内死于呼吸衰竭，并伴有严重的肺气肿。NPAS3杂合子(Het)存活，但有不同程度的肺气肿和/或反应性呼吸道疾病。我们不知道不同年龄的肺表型是否存在差异(即肺气肿随着年龄的增长而恶化)，也不知道不同动物之间是否存在个体差异。微型CT扫描将能够分析不同时间点的单个动物的肺组织。对一只NPAS3-Het和一只野生型窝产仔的比较是一项重要的中试实验，用于竞争更新我们的赠款。

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. We cloned npas3 as the mouse homolog of a Drosophila gene, trachealess, the null mutant of which completely lacks a respiratory system. 90% of our npas3-null mice die within a few hours after birth of respiratory failure, with severely emphysematous lungs. The npas3 heterozygotes (Het) live, but have varying degrees of emphysema and/or reactive airways disease. We don't know whether there are differences in the lung phenotype at different ages (i.e. worsening of emphysema with aging) or whether there is individual variability between different animals. Micro-CT scans will permit analysis of the lung tissue of individual animals at different time points. Comparison of one npas3-Het and one wild-type littermate is an important pilot experiment for the competing renewal of our grant.