Fernandez H L, Stiles J R, Donoso J A
Muscle Nerve. 1986 Jun;9(5):399-406. doi: 10.1002/mus.880090504.
Acetylcholinesterase (AChE) molecular forms in muscle biopsies from control and amyotrophic lateral sclerosis (ALS) patients were extracted under low (G: globular forms) and high (A: asymmetric forms) ionic strength conditions and were evaluated by velocity sedimentation analysis. Total AChE activity in endplate-containing ALS muscle sections was reduced by an average of 65% of control muscle levels. This decrement resulted from an almost complete disappearance of 9.5S (G4) and 8.0S (A4) AChE and significant decreases in the 3.8S (G1), 12.1S (A8), and 15.8S (A12) forms (66%, 9%, and 25% of control, respectively). In most of the ALS biopsies examined, ultrastructural-cytochemical analysis revealed large reductions in AChE reaction product of both synaptic infoldings (extracellular) and sarcoplasmic reticulum (intracellular) of the muscles' motor endplate regions. These data are compatible with the view that alterations observed in AChE forms from ALS muscles are related to disturbances in the normal "trophic" interactions between nerve and muscle.
在低离子强度条件（G：球状形式）和高离子强度条件（A：不对称形式）下，从对照者和肌萎缩侧索硬化症（ALS）患者的肌肉活检中提取乙酰胆碱酯酶（AChE）分子形式，并通过速度沉降分析进行评估。含有终板的ALS肌肉切片中的总AChE活性平均降低至对照肌肉水平的65%。这种降低是由于9.5S（G4）和8.0S（A4）AChE几乎完全消失，以及3.8S（G1）、12.1S（A8）和15.8S（A12）形式显著减少（分别为对照的66%、9%和25%）所致。在大多数检查的ALS活检样本中，超微结构细胞化学分析显示，肌肉运动终板区域的突触褶皱（细胞外）和肌浆网（细胞内）的AChE反应产物均大幅减少。这些数据与以下观点一致，即ALS肌肉中观察到的AChE形式改变与神经和肌肉之间正常“营养”相互作用的紊乱有关。
