Wang Zhi-Hao, Liu Pai, Liu Xia, Manfredsson Fredric P, Sandoval Ivette M, Yu Shan Ping, Wang Jian-Zhi, Ye Keqiang
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA; Department of Pathophysiology, Key Laboratory of Ministry of Education of Neurological Diseases, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Emory College of Arts and Sciences, Emory University, Atlanta, GA 30322, USA.
Mol Cell. 2017 Sep 7;67(5):812-825.e5. doi: 10.1016/j.molcel.2017.07.018. Epub 2017 Aug 17.
Delta-secretase, a lysosomal asparagine endopeptidase (AEP), simultaneously cleaves both APP and tau, controlling the onset of pathogenesis of Alzheimer's disease (AD). However, how this protease is post-translationally regulated remains unclear. Here we report that serine-arginine protein kinase 2 (SRPK2) phosphorylates delta-secretase and enhances its enzymatic activity. SRPK2 phosphorylates serine 226 on delta-secretase and accelerates its autocatalytic cleavage, leading to its cytoplasmic translocation and escalated enzymatic activities. Delta-secretase is highly phosphorylated in human AD brains, tightly correlated with SRPK2 activity. Overexpression of a phosphorylation mimetic (S226D) in young 3xTg mice strongly promotes APP and tau fragmentation and facilitates amyloid plaque deposits and neurofibrillary tangle (NFT) formation, resulting in cognitive impairment. Conversely, viral injection of the non-phosphorylatable mutant (S226A) into 5XFAD mice decreases APP and tau proteolytic cleavage, attenuates AD pathologies, and reverses cognitive defects. Our findings support that delta-secretase phosphorylation by SRPK2 plays a critical role in aggravating AD pathogenesis.
δ-分泌酶是一种溶酶体天冬酰胺内肽酶(AEP),它能同时切割淀粉样前体蛋白(APP)和tau蛋白,从而控制阿尔茨海默病(AD)发病机制的起始。然而,这种蛋白酶在翻译后是如何被调控的仍不清楚。在此我们报告,丝氨酸-精氨酸蛋白激酶2(SRPK2)使δ-分泌酶磷酸化并增强其酶活性。SRPK2使δ-分泌酶上的丝氨酸226磷酸化,并加速其自催化切割,导致其向细胞质转位并提升酶活性。δ-分泌酶在人类AD大脑中高度磷酸化,这与SRPK2活性密切相关。在年轻的3xTg小鼠中过表达磷酸化模拟物(S226D)强烈促进APP和tau蛋白片段化,并促进淀粉样斑块沉积和神经原纤维缠结(NFT)形成,导致认知障碍。相反,将不可磷酸化突变体(S226A)通过病毒注射到5XFAD小鼠体内,可减少APP和tau蛋白的蛋白水解切割,减轻AD病理,并逆转认知缺陷。我们的研究结果支持,SRPK2介导的δ-分泌酶磷酸化在加重AD发病机制中起关键作用。