Giorgi Filippo S, Ryskalin Larisa, Ruffoli Riccardo, Biagioni Francesca, Limanaqi Fiona, Ferrucci Michela, Busceti Carla L, Bonuccelli Ubaldo, Fornai Francesco
Section of Neurology, Pisa University Hospital, Department of Clinical and Experimental Medicine, University of PisaPisa, Italy.
Department of Translational Research and New Technologies in Medicine and Surgery, University of PisaPisa, Italy.
Front Neuroanat. 2017 Sep 19;11:80. doi: 10.3389/fnana.2017.00080. eCollection 2017.
Alzheimer's Disease (AD) features the accumulation of β-amyloid and Tau aggregates, which deposit as extracellular plaques and intracellular neurofibrillary tangles (NFTs), respectively. Neuronal Tau aggregates may appear early in life, in the absence of clinical symptoms. This occurs in the brainstem reticular formation and mostly within Locus Coeruleus (LC), which is consistently affected during AD. LC is the main source of forebrain norepinephrine (NE) and it modulates a variety of functions including sleep-waking cycle, alertness, synaptic plasticity, and memory. The iso-dendritic nature of LC neurons allows their axons to spread NE throughout the whole forebrain. Likewise, a prion-like hypothesis suggests that Tau aggregates may travel along LC axons to reach out cortical neurons. Despite this timing is compatible with cross-sectional studies, there is no actual evidence for a causal relationship between these events. In the present mini-review, we dedicate special emphasis to those various mechanisms that may link degeneration of LC neurons to the onset of AD pathology. This includes the hypothesis that a damage to LC neurons contributes to the onset of dementia due to a loss of neuroprotective effects or, even the chance that, LC degenerates independently from cortical pathology. At the same time, since LC neurons are lost in a variety of neuropsychiatric disorders we considered which molecular mechanism may render these brainstem neurons so vulnerable.
阿尔茨海默病(AD)的特征是β-淀粉样蛋白和Tau聚集体的积累,它们分别以细胞外斑块和细胞内神经原纤维缠结(NFTs)的形式沉积。神经元Tau聚集体可能在生命早期出现,且无临床症状。这种情况发生在脑干网状结构中,主要在蓝斑(LC)内,而蓝斑在AD过程中始终会受到影响。蓝斑是前脑去甲肾上腺素(NE)的主要来源,它调节多种功能,包括睡眠-觉醒周期、警觉性、突触可塑性和记忆。蓝斑神经元的等树突性质使其轴突能够将去甲肾上腺素扩散到整个前脑。同样,一种朊病毒样假说是,Tau聚集体可能沿着蓝斑轴突传播以接触皮质神经元。尽管这个时间顺序与横断面研究相符,但尚无这些事件之间因果关系的实际证据。在本综述中,我们特别强调那些可能将蓝斑神经元变性与AD病理发生联系起来的各种机制。这包括这样的假说,即蓝斑神经元的损伤由于神经保护作用的丧失而导致痴呆的发生,甚至包括蓝斑独立于皮质病理而退化的可能性。同时,由于蓝斑神经元在多种神经精神疾病中都会丢失,我们考虑了哪些分子机制可能使这些脑干神经元如此脆弱。