Lin Tong, Tjernberg Lars O, Schedin-Weiss Sophia
Division of Neurogeriatrics, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, 17164 Stockholm, Sweden.
Biomedicines. 2021 Jul 10;9(7):801. doi: 10.3390/biomedicines9070801.
Alzheimer's disease (AD) is the most common type of dementia, contributing to 60-80% of cases. It is a neurodegenerative disease that usually starts symptomless in the first two to three decades and then propagates into a long-term, irreversible disease, resulting in the progressive loss of memory, reasoning, abstraction and language capabilities. It is a complex disease, involving a large number of entangled players, and there is no effective treatment to cure it or alter its progressive course. Therefore, a thorough understanding of the disease pathology and an early diagnosis are both necessary. AD has two significant pathological hallmarks: extracellular senile plaques composed of amyloid β-peptide (Aβ) and intracellular neurofibrillary tangles composed of hyperphosphorylated tau protein, and the aggregation of Aβ, which starts in earlier stages, is usually claimed to be the primary cause of AD. Secretases that cleave Aβ precursor protein (APP) and produce neurotoxic Aβ reside in distinct organelles of the cell, and current concepts suggest that APP moves between distinct intracellular compartments. Obviously, APP transport and processing are intimately related processes that cannot be dissociated from each other, and, thus, how and where APP is transported determines its processing fate. In this review, we summarize critical mechanisms underlying neuronal APP transport, which we divide into separate parts: (1) secretory pathways and (2) endocytic and autophagic pathways. We also include two lipoprotein receptors that play essential roles in APP transport: sorting-related receptor with A-type repeats and sortilin. Moreover, we consider here some major disruptions in the neuronal transport of APP that contribute to AD physiology and pathology. Lastly, we discuss current methods and technical difficulties in the studies of APP transport.
阿尔茨海默病(AD)是最常见的痴呆类型,占病例的60 - 80%。它是一种神经退行性疾病,通常在最初的二三十年内无症状开始,然后发展为一种长期的、不可逆的疾病,导致记忆、推理、抽象和语言能力逐渐丧失。这是一种复杂的疾病,涉及大量相互纠缠的因素,并且没有有效的治疗方法来治愈它或改变其进展过程。因此,全面了解疾病病理和早期诊断都很有必要。AD有两个显著的病理特征:由淀粉样β肽(Aβ)组成的细胞外老年斑和由过度磷酸化的tau蛋白组成的细胞内神经原纤维缠结,通常认为在早期阶段开始的Aβ聚集是AD的主要原因。切割Aβ前体蛋白(APP)并产生神经毒性Aβ的分泌酶存在于细胞的不同细胞器中,目前的观点认为APP在不同的细胞内区室之间移动。显然,APP的运输和加工是密切相关且不可分割的过程,因此,APP如何以及在何处运输决定了其加工命运。在这篇综述中,我们总结了神经元APP运输的关键机制,我们将其分为不同部分:(1)分泌途径和(2)内吞和自噬途径。我们还包括两种在APP运输中起重要作用的脂蛋白受体:具有A型重复序列的分选相关受体和sortilin。此外,我们在此考虑了一些导致AD生理和病理的APP神经元运输的主要破坏因素。最后,我们讨论了APP运输研究中的当前方法和技术困难。