Zhao Li-Chun, Yang Bo, Wang Rengang, Lipton Stuart A, Zhang Dongxian
Center for Neuroscience, Aging and Stem Cell Research, Burnham Institute for Medical Research, La Jolla, California, USA.
School of Pharmacy, Jilin University, Changchun, China.
Neuroreport. 2010 Jan 6;21(1):14-18. doi: 10.1097/WNR.0b013e328330dcca.
All botulinum toxins (BoNTs, types A-G) inhibit synaptic transmitter release from motoneurons, and thus result in respiratory arrest and death. Rapid treatment with anti-BoNT antibodies can prevent progression, but recovery still requires weeks on a ventilator. Even after recovery, there is a potential for persistent fatigue in some cases of botulism even years after the insult, possibly because of motoneuron dropout for previously unknown reasons. Unique among BoNTs, the C-type (BoNT/C) cleaves two proteins involved in neurotransmitter release, syntaxin and SNAP-25, and induces apoptotic cell death in cultured cerebellar neurons. It is not clear, however, whether BoNT/C also affects neurons that encounter toxin in vivo, namely motoneurons. Here, we provide experimental evidence that BoNT/C causes a slow degeneration of motoneurons both in vitro and in vivo. This novel form of BoNT/C-induced cell death may require new treatment strategies.
所有肉毒杆菌毒素(BoNTs,A - G型)均抑制运动神经元释放突触递质,从而导致呼吸骤停和死亡。用抗BoNT抗体进行快速治疗可防止病情进展,但恢复仍需在呼吸机上维持数周。即使在恢复后,肉毒中毒的某些病例即使在中毒数年之后仍有可能出现持续疲劳,这可能是由于此前未知的原因导致运动神经元缺失。在BoNTs中独一无二的是,C型(BoNT/C)可切割参与神经递质释放的两种蛋白质,即 syntaxin 和 SNAP - 25,并在培养的小脑神经元中诱导凋亡性细胞死亡。然而,尚不清楚BoNT/C是否也会影响体内接触毒素的神经元,即运动神经元。在此,我们提供实验证据表明,BoNT/C在体外和体内均会导致运动神经元的缓慢退化。这种由BoNT/C诱导的新型细胞死亡可能需要新的治疗策略。