Centre Mémoire de Ressources et de Recherche Paris Nord Ile de France.
Brain Pathol. 2012 Mar;22(2):219-29. doi: 10.1111/j.1750-3639.2011.00520.x. Epub 2011 Sep 16.
The neuropathological hallmarks of Alzheimer's disease (AD) include senile plaques made of Aβ peptide, neurofibrillary tangles containing hyperphosphorylated tau protein and neuronal loss. The pro-apoptotic kinase PKR can be activated by Aβ and can phosphorylate tau protein via GSK3β kinase activation. The activated form of PKR (pPKR) accumulates in affected neurons and could participate in neuronal degeneration in AD. The mechanism of abnormal PKR activation in AD is not elucidated but could be linked to the PKR activator PACT. PACT stainings, and levels were assessed in the brains of AD patients and in APP/PS1 knock-in transgenic mice and in cell cultures exposed to stresses. We showed that PACT and pPKR colocalizations are enhanced in AD brains. Their levels are increased and correlated in AD and APP/PS1 knock-in mice brains. In human neuroblastoma cells exposed to Aβ, tunicamycin or H2O2, PACT and pPKR concentrations are increased. PACT then PKR inhibitions indicate that PACT is upstream of PKR activation. Our findings demonstrate that PACT levels are enhanced in AD brains and could partly be caused by the action of Aβ. In addition, PACT participates in PKR activation. The PACT-PKR pathway represents a potential link between Aβ accumulation, PKR activation and tau phosphorylation.
阿尔茨海默病(AD)的神经病理学特征包括由 Aβ 肽组成的老年斑、含有过度磷酸化 tau 蛋白的神经原纤维缠结和神经元丢失。促凋亡激酶 PKR 可被 Aβ 激活,并通过 GSK3β 激酶激活磷酸化 tau 蛋白。PKR 的激活形式(pPKR)在受影响的神经元中积累,可能参与 AD 中的神经元退化。AD 中异常 PKR 激活的机制尚不清楚,但可能与 PKR 激活剂 PACT 有关。我们评估了 AD 患者和 APP/PS1 基因敲入转基因小鼠以及暴露于应激的细胞培养物中 PACT 的染色和水平。我们表明,AD 大脑中 PACT 和 pPKR 的共定位增强。它们的水平在 AD 和 APP/PS1 基因敲入小鼠大脑中增加并相关。在暴露于 Aβ、衣霉素或 H2O2 的人神经母细胞瘤细胞中,PACT 和 pPKR 浓度增加。然后 PKR 抑制表明 PACT 是 PKR 激活的上游。我们的研究结果表明,PACT 水平在 AD 大脑中增强,部分可能是由 Aβ 的作用引起的。此外,PACT 参与了 PKR 的激活。PACT-PKR 途径代表了 Aβ 积累、PKR 激活和 tau 磷酸化之间的潜在联系。