Axonal transport and neurological disease.,Nature Reviews Neurology

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Axonal transport and neurological disease.
Nature Reviews Neurology ( IF 28.2 ) Pub Date : 2019-09-26 , DOI: 10.1038/s41582-019-0257-2
James N Sleigh _{1,

2} , Alexander M Rossor ₁ , Alexander D Fellows ₁ , Andrew P Tosolini ₁ , Giampietro Schiavo _{1,

2,

3}

Affiliation

Axonal transport is the process whereby motor proteins actively navigate microtubules to deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is widely regarded as essential for nerve development, function and survival. Mutations in genes encoding key components of the transport machinery, including motor proteins, motor adaptors and microtubules, have been discovered to cause neurological disease. Moreover, disruptions in axonal cargo trafficking have been extensively reported across a wide range of nervous system disorders. However, whether these impairments have a major causative role in, are contributing to or are simply a consequence of neuronal degeneration remains unclear. Therefore, the fundamental relevance of defective trafficking along axons to nerve dysfunction and pathology is often debated. In this article, we review the latest evidence emerging from human and in vivo studies on whether perturbations in axonal transport are indeed integral to the pathogenesis of neurological disease.

中文翻译：

轴突运输和神经系统疾病。

轴突运输是运动蛋白主动导航微管以将不同的货物（例如细胞器）从轴突的一端传递到另一端的过程，被广泛认为对神经发育、功能和生存至关重要。已发现编码运输机制关键成分（包括运动蛋白、运动接头和微管）的基因突变会导致神经系统疾病。此外，在广泛的神经系统疾病中广泛报道了轴突货物运输的中断。然而，这些损伤是否在神经元变性中起主要的致病作用、促成或仅仅是神经元变性的结果仍不清楚。因此，沿轴突运输缺陷与神经功能障碍和病理学的基本相关性经常受到争论。

更新日期：2019-09-26

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