Dahlström Märta, Nordvall Gunnar, Sundström Erik, Åkesson Elisabet, Tegerstedt Gunilla, Eriksdotter Maria, Forsell Pontus
Division of Clinical Geriatrics, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Huddinge, Sweden.
AlzeCure Foundation, Huddinge, Sweden.
Eur J Neurosci. 2019 Nov;50(9):3487-3501. doi: 10.1111/ejn.14513. Epub 2019 Aug 1.
Nerve growth factor (NGF) is an essential neurotrophic factor for the development and maintenance of the central and the peripheral nervous system. NGF deficiency in the basal forebrain precedes degeneration of basal forebrain cholinergic neurons in Alzheimer's disease, contributing to memory decline. NGF mediates neurotrophic support via its high-affinity receptor, the tropomyosin-related kinase A (TrkA) receptor, and mediates mitogenic and differentiation signals via the extracellular signal-regulated protein kinases 1 and 2 (ERK1/2). However, the molecular mechanisms underlying the different NGF/TrkA/ERK signalling pathways are far from clear. In this study, we have investigated the role of human NGF and three NGF mutants, R100E, W99A and K95A/Q96A, their ability to activate TrkA or ERK1/2, and their ability to induce proliferation or differentiation in human foetal dorsal root ganglion (DRG) neurons or in PC12 cells. We show that the R100E mutant was significantly more potent than NGF itself to induce proliferation and differentiation, and significantly more potent in activation of ERK1/2 in DRG neurons. The W99A and K95A/Q96A mutants, on the other hand, were less effective than the wild-type protein. An unexpected finding was the high efficacy of the K95A/Q96A mutant to activate TrkA and to induce differentiation of DRG neurons at elevated concentrations. These data demonstrate an NGF mutant with improved neurotrophic properties in primary human neuronal cells. The R100E mutant represents an interesting candidate for further drug development in Alzheimer's disease and other neurodegenerative disorders.
神经生长因子(NGF)是中枢和外周神经系统发育与维持所必需的神经营养因子。阿尔茨海默病中,基底前脑的NGF缺乏先于基底前脑胆碱能神经元的退化,导致记忆衰退。NGF通过其高亲和力受体——原肌球蛋白相关激酶A(TrkA)受体介导神经营养支持,并通过细胞外信号调节蛋白激酶1和2(ERK1/2)介导有丝分裂和分化信号。然而,不同的NGF/TrkA/ERK信号通路的分子机制尚不清楚。在本研究中,我们研究了人NGF及其三个突变体R100E、W99A和K95A/Q96A的作用,它们激活TrkA或ERK1/2的能力,以及它们在人胎儿背根神经节(DRG)神经元或PC12细胞中诱导增殖或分化的能力。我们发现,R100E突变体在诱导DRG神经元增殖和分化方面比NGF本身显著更有效,在激活DRG神经元中的ERK1/2方面也显著更有效。另一方面,W99A和K95A/Q96A突变体比野生型蛋白效果更差。一个意外的发现是,K95A/Q96A突变体在浓度升高时激活TrkA和诱导DRG神经元分化的效率很高。这些数据证明了一种在原代人神经元细胞中具有改善的神经营养特性的NGF突变体。R100E突变体是阿尔茨海默病和其他神经退行性疾病进一步药物开发的一个有趣候选物。