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膜联蛋白A6膜修复蛋白在阿尔茨海默病模型小鼠中可预防淀粉样蛋白诱导的营养不良性神经突和tau蛋白磷酸化。

Annexin A6 membrane repair protein protects against amyloid-induced dystrophic neurites and tau phosphorylation in Alzheimer's disease model mice.

作者信息

Sadleir Katherine R, Gomez Karen P, Edwards Abigail E, Patel Armana J, Ley Makenna L, Khatri Ammaarah W, Guo Joanna, Mahesh Shreya, Watkins Elyse A, Popovic Jelena, Karunakaran Devi Krishna Priya, Prokopenko Dmitry, Tanzi Rudolph E, Bustos Bernabe, Lubbe Steven J, Demonbruen Alexis R, McNally Elizabeth M, Vassar Robert

机构信息

Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL, 60611, USA.

Department of Neurology, Genetics and Aging Research Unit and the McCance Center for Brain Health, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA, USA.

出版信息

Acta Neuropathol. 2025 May 24;149(1):51. doi: 10.1007/s00401-025-02888-1.

Abstract

In Alzheimer's disease, accumulation of amyloid-β (Aβ) peptide is thought to cause formation of neurofibrillary tangles composed of hyperphosphorylated tau protein, which correlates with neuronal loss and cognitive impairment, but the mechanism linking Aβ and tau pathologies is unknown. Dystrophic neurites, which surround Aβ plaques and accumulate phosphorylated tau and other proteins, may play a role in seeding and spreading of pathologic tau. Here, we investigate the novel hypothesis that improved membrane repair capacity decreases dystrophic neurite formation by protecting axons from Aβ-induced membrane damage. Using a ratiometric calcium sensor and a FRET-based calpain cleavage sensor, we demonstrate that dystrophic neurites in 5XFAD mice have elevated resting calcium levels and calpain activity because of putative membrane damage. Annexin A6, a plasma membrane repair in muscle and neurons, is present at plasma membrane of neurons and dystrophic neurites in murine and human brains. Overexpression of annexin A6 in brains of 5XFAD mice decreased size and quantity of dystrophic neurites and accumulation of phospho-tau181, an early biomarker of amyloid pathology. Phospho-tau231, another early amyloid biomarker, and phosphorylated of tau kinases, c-jun N-terminal kinase (JNK) and Calmodulin Kinase II (CaMKII) accumulate in dystrophic neurites in the brains of amyloid pathology mice and humans with AD, suggesting that dystrophic neurites are sites of active tau phosphorylation. Overexpression of dominant-negative annexin A6 in 5XFAD mice increased dystrophic neurites and phospho-tau181. Intracerebral injection of recombinant annexin A6 in 5XFAD and APP-NLGF knock-in mice resulted in localization of recombinant A6 to membranes of dystrophic neurites, suggesting therapeutic potential of recombinant annexin A6 for AD. In conclusion, dystrophic neurites have Aβ-induced membrane damage characterized by calcium elevation, calpain activation, and accumulation of tau kinases and phosphorylated tau. Overexpression of annexin A6 reduces dystrophic neurites and phospho-tau accumulation, suggesting that annexin A6-mediated membrane repair may represent a novel therapeutic approach for AD.

摘要

在阿尔茨海默病中,淀粉样β(Aβ)肽的积累被认为会导致由高度磷酸化的tau蛋白组成的神经原纤维缠结的形成,这与神经元丢失和认知障碍相关,但连接Aβ和tau病理学的机制尚不清楚。营养不良性神经突围绕着Aβ斑块并积累磷酸化的tau蛋白和其他蛋白质,可能在病理性tau的播种和传播中起作用。在这里,我们研究了一个新的假设,即提高膜修复能力可通过保护轴突免受Aβ诱导的膜损伤来减少营养不良性神经突的形成。使用比率钙传感器和基于荧光共振能量转移(FRET)的钙蛋白酶裂解传感器,我们证明5XFAD小鼠中的营养不良性神经突由于假定的膜损伤而具有升高的静息钙水平和钙蛋白酶活性。膜联蛋白A6是肌肉和神经元中的一种质膜修复蛋白,存在于小鼠和人类大脑中神经元和营养不良性神经突的质膜上。在5XFAD小鼠的大脑中过表达膜联蛋白A6可减少营养不良性神经突的大小和数量以及磷酸化tau181的积累,磷酸化tau181是淀粉样病理的一种早期生物标志物。另一种早期淀粉样生物标志物磷酸化tau231以及tau激酶、c-jun氨基末端激酶(JNK)和钙调蛋白激酶II(CaMKII)的磷酸化在淀粉样病理小鼠和患有阿尔茨海默病的人类大脑中的营养不良性神经突中积累,这表明营养不良性神经突是活跃的tau磷酸化位点。在5XFAD小鼠中过表达显性负性膜联蛋白A6会增加营养不良性神经突和磷酸化tau181。在5XFAD和APP-NLGF基因敲入小鼠中脑内注射重组膜联蛋白A6导致重组A6定位于营养不良性神经突的膜上,这表明重组膜联蛋白A6对阿尔茨海默病具有治疗潜力。总之,营养不良性神经突具有Aβ诱导的膜损伤,其特征为钙升高、钙蛋白酶激活以及tau激酶和磷酸化tau的积累。膜联蛋白A6的过表达减少了营养不良性神经突和磷酸化tau的积累,这表明膜联蛋白A6介导的膜修复可能代表了一种治疗阿尔茨海默病的新方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/575a/12103342/691a23a70493/401_2025_2888_Fig1_HTML.jpg

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