Bertone Nicolas Ivan, Groisman Ayelén Ivana, Mazzone Graciela Lujan, Cano Raquel, Tabares Lucia, Uchitel Osvaldo Daniel
Instituto de Fisiología, Biología Molecular y Neurociencias (IFIBYNE-UBA-CONICET) and Departamento de Fisiología, Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Ciudad Universitaria, Buenos Aires C1428EHA, Argentina.
Laboratorios de Investigación aplicada en Neurociencias (LIAN)-Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia (FLENI), CONICET, Buenos Aires, Argentina.
Synapse. 2017 Dec;71(12). doi: 10.1002/syn.22009. Epub 2017 Sep 21.
Acetazolamide (AZ), a molecule frequently used to treat different neurological syndromes, is an inhibitor of the carbonic anhydrase (CA), an enzyme that regulates pH inside and outside cells. We combined fluorescent FM styryl dyes and electrophysiological techniques at ex vivo levator auris longus neuromuscular junctions (NMJs) from mice to investigate the modulation of synaptic transmission and vesicle recycling by AZ. Transmitter release was minimally affected by AZ, as evidenced by evoked and spontaneous end-plate potential measurements. However, optical evaluation with FM-styryl dyes of vesicle exocytosis elicited by 50 Hz stimuli showed a strong reduction in fluorescence loss in AZ treated NMJ, an effect that was abolished by bathing the NMJ in Hepes. The remaining dye was quenched by bromophenol, a small molecule capable of diffusing inside vesicles. Furthermore, in transgenic mice expressing Synaptophysin-pHluorin (SypHy), the fluorescence responses of motor nerve terminals to a 50 Hz train of stimuli was decrease to a 50% of controls in the presence of AZ. Immunohistochemistry experiments to evaluate the state of the Myosin light chain kinase (MLCK), an enzyme involved in vesicle recycling, demonstrated that MLCK phosphorylation was much stronger in the presence than AZ than in its absence in 50 Hz stimulated NMJs. We postulate that AZ, via cytosol acidification and activation of MLCK, shifts synaptic vesicle recycling to a fast (kiss-and-run) mode, which changes synaptic performance. These changes may contribute to the therapeutic action reported in many neurological syndromes like ataxia, epilepsy, and migraine.
乙酰唑胺(AZ)是一种常用于治疗不同神经综合征的分子,它是碳酸酐酶(CA)的抑制剂,碳酸酐酶是一种调节细胞内外pH值的酶。我们将荧光FM苯乙烯基染料与电生理技术相结合,在小鼠离体的耳长肌神经肌肉接头(NMJ)处,研究AZ对突触传递和囊泡循环的调节作用。通过诱发和自发终板电位测量表明,递质释放受AZ的影响最小。然而,用FM苯乙烯基染料对50Hz刺激引发的囊泡胞吐作用进行光学评估发现,在AZ处理的NMJ中荧光损失明显减少,将NMJ置于Hepes中可消除这种效应。剩余的染料被溴酚淬灭,溴酚是一种能够扩散到囊泡内部的小分子。此外,在表达突触素-pHluorin(SypHy)的转基因小鼠中,在存在AZ的情况下,运动神经末梢对50Hz刺激序列的荧光反应降低至对照的50%。免疫组织化学实验评估参与囊泡循环的肌球蛋白轻链激酶(MLCK)的状态,结果表明,在50Hz刺激的NMJ中,存在AZ时MLCK的磷酸化比不存在时更强。我们推测,AZ通过胞质溶胶酸化和MLCK的激活,将突触囊泡循环转变为快速(亲吻-逃离)模式,从而改变突触性能。这些变化可能有助于解释在许多神经综合征如共济失调、癫痫和偏头痛中报道的治疗作用。