Department of Cell Biology and Neuroscience Program, University of Miami Miller School of Medicine, Miami, FL, USA.
Neurosci Lett. 2020 Sep 14;735:135157. doi: 10.1016/j.neulet.2020.135157. Epub 2020 Jun 12.
Acetylcholinesterase (AChE) is an essential enzymatic component of the neuromuscular junction where it is responsible for terminating neurotransmission by the cholinergic motor neurons. The enzyme at the neuromuscular junction (NMJ) is contributed primarily by the skeletal muscle where it is produced at higher levels in the post-synaptic region of the fibers. The major form of AChE at the NMJ is a large asymmetric form consisting of three tetramers covalently attached to a three-stranded collagen-like tail which is responsible for anchoring it to the synaptic basal lamina. Its location and expression is regulated to a large extent by the motor neurons and occurs at the transcriptional, translational and post-translational levels. While its expression can be quite rapid in tissue cultured cells, its half-life in vivo appears to be quite long, about three weeks, although more rapidly turning over pools have been described. Finally the essential nature of this enzyme is underscored by the fact that no naturally occurring null mutations of the catalytic subunit have been described in higher organisms and the few dozen humans carrying mutations in the collagen tail responsible for anchoring the enzyme at the NMJ are severely affected.
乙酰胆碱酯酶（AChE）是神经肌肉接头的一种重要酶类成分，负责终止胆碱能运动神经元的神经递质传递。该酶在神经肌肉接头（NMJ）主要由骨骼肌贡献，其在纤维的突触后区域产生更高水平。NMJ 上的主要 AChE 形式是一种大型不对称形式，由三个四聚体通过共价键附着在三股胶原样尾巴上，负责将其锚定在突触基底层上。其位置和表达在很大程度上受到运动神经元的调节，并在转录、翻译和翻译后水平发生。虽然它在组织培养细胞中的表达可以非常迅速，但它在体内的半衰期似乎相当长，约为三周，尽管已经描述了更快速周转的池。最后，这种酶的本质是由这样一个事实所强调的，即在高等生物中尚未描述出催化亚基的自然缺失突变，而少数几十个人携带负责将酶锚定在 NMJ 的胶原尾部的突变，这些人受到严重影响。
