ORGANIZATION AND FUNCTIONS OF THE NUCLEAR LAMINA

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 研究中心，而研究中心不一定是研究者所在的机构。 Hutchinson-Guilford早衰症是由核纤层蛋白A的突变形式引起的，该突变形式在其C末端区域包含内部缺失。 这阻止了通常发生的核纤层蛋白A的法尼基化C-末端的蛋白水解去除，并导致功能的毒性获得。为了了解这种疾病的分子基础，我们正在进行蛋白质组学分析的核膜分离的小鼠肝脏突变，阻止正常去除核纤层蛋白A法尼基化的C-末端。

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. Hutchinson-Guilford progeria is caused by a mutant form of lamin A that contains an internal deletion in its C-terminal region. This prevents the proteolytic removal of the farnesylated C-terminus of lamin A that normally takes place, and leads to a toxic gain of function. To understand the molecular basis of this disease, we are carrying out proteomic analysis of nuclear envelopes isolated from the livers of mice with mutations that prevent normal removal of the lamin A farnesylated C-terminus.