Kakaroubas Nicholas, Brennan Samuel, Keon Matthew, Saksena Nitin K
Neurodegenerative Disease Section, Iggy Get Out, 19A Boundary Street, Darlinghurst NSW 2010, Sydney, Australia.
School of Biotechnology and Biomolecular Sciences, University of New South Wales (University of NSW), Chancellery Walk, Kensington NSW 2033, Sydney, Australia.
Neurosci J. 2019 Jul 10;2019:2537698. doi: 10.1155/2019/2537698. eCollection 2019.
The blood-brain barrier (BBB) and the blood-spinal cord barrier (BSCB) are responsible for controlling the microenvironment within neural tissues in humans. These barriers are fundamental to all neurological processes as they provide the extreme nutritional demands of neural tissue, remove wastes, and maintain immune privileged status. Being a semipermeable membrane, both the BBB and BSCB allow the diffusion of certain molecules, whilst restricting others. In amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases, these barriers become hyperpermeable, allowing a wider variety of molecules to pass through leading to more severe and more rapidly progressing disease. The intention of this review is to discuss evidence that BBB hyperpermeability is potentially a disease driving feature in ALS and other neurodegenerative diseases. The various biochemical, physiological, and genomic factors that can influence BBB permeability in ALS and other neurodegenerative diseases are also discussed, in addition to novel therapeutic strategies centred upon the BBB.
血脑屏障（BBB）和血脊髓屏障（BSCB）负责控制人类神经组织内的微环境。这些屏障对于所有神经学过程至关重要，因为它们满足神经组织极高的营养需求、清除废物并维持免疫豁免状态。作为半透膜，血脑屏障和血脊髓屏障允许某些分子扩散，同时限制其他分子。在肌萎缩侧索硬化症（ALS）和其他神经退行性疾病中，这些屏障会变得通透性过高，使得更多种类的分子能够通过，从而导致疾病更加严重且进展更快。本综述旨在讨论血脑屏障通透性过高可能是ALS和其他神经退行性疾病的一种疾病驱动特征的证据。此外，还将讨论可能影响ALS和其他神经退行性疾病中血脑屏障通透性的各种生化、生理和基因组因素，以及以血脑屏障为中心的新型治疗策略。
