The peptidyl-prolyl cis-trans isomerase (PPIase) Pin1 modulates the activity of a range of target proteins involved in the cell cycle, transcription, translation, endocytosis, and apoptosis by facilitating dephosphorylation of phosphorylated serine or threonine residue preceding a proline (p-Ser/Thr-Pro) motifs catalyzed by phosphatases specific for the trans conformations. Pin1 targets include the neuronal microtubule-associated protein tau, whose dephosphorylation restores its ability to stabilize microtubules. We, and others, have shown that tau hyperphosphorylation in the neurofibrillary tangles (NFTs) of Alzheimer disease (AD) is associated with redirection of the predominantly nuclear Pin1 to the cytoplasm and with Pin1 shortfalls throughout subcellular compartments. As nuclear Pin1 depletion causes apoptosis, shortfalls in regard to both nuclear and p-tau targets may contribute to neuronal dysfunction. We report here that similar Pin1 redistribution and shortfalls occur in frontotemporal dementias (FTDs) characterized by abnormal protein aggregates of tau and other cytoskeletal proteins. This may be a unifying, contributory factor towards neuronal death in these dementias.

译文

肽基-脯氨酰顺式反式异构酶 (PPIase) Pin1调节一系列参与细胞周期,转录,翻译,内吞作用的靶蛋白的活性,通过促进对反式构象特异的磷酸酶催化的脯氨酸 (p-Ser/Thr-Pro) 基序之前的磷酸化丝氨酸或苏氨酸残基的去磷酸化而凋亡。Pin1靶标包括神经元微管相关蛋白tau,其去磷酸化恢复其稳定微管的能力。我们和其他人已经表明,阿尔茨海默氏病 (AD) 的神经原纤维缠结 (nft) 中的tau过度磷酸化与主要的核Pin1重定向到细胞质以及整个亚细胞区室的Pin1不足有关。由于核Pin1耗竭会导致细胞凋亡,因此核和p-tau靶标的不足可能会导致神经元功能障碍。我们在此报告说,以tau蛋白和其他细胞骨架蛋白的异常蛋白聚集体为特征的额颞叶痴呆 (FTDs) 中也发生了类似的Pin1再分配和不足。这可能是导致这些痴呆症神经元死亡的统一因素。

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