Biomolecular Dynamics Laboratory, Department of Biomolecular Networks, Graduate School of Frontier Biosciences, Osaka University, Osaka, Japan.
Biochem Biophys Res Commun. 2013 Oct 25;440(3):359-63. doi: 10.1016/j.bbrc.2013.08.052. Epub 2013 Aug 26.
Neurodegeneration causes dysfunction and degeneration of neurons and is triggered by various factors including genetic defects, free radicals, injury, and glutamate excitotoxicity. Among those, glutamate excitotoxicity is implicated in chronic disorders including AD and ALS, and in acute insults in the CNS including traumatic brain injury. Neurological disorders show hallmark morphological abnormalities such as axon degeneration and cell body death. The molecular mechanisms underlying excitotoxicity-induced neurodegeneration are complex and deciphering a molecular mechanism from one angle is beneficial to understand the process, however, still difficult to develop strategies to suppress excitotoxicity-induced degeneration due to existence of other mechanisms. Thus, directly identifying compounds that can modulate excitotoxicity-induced neurodegeneration and subsequently clarifiying the molecular mechanism is a valid approach to develop effective strategies to suppress neurodegeneration. We searched for compounds that can suppress excitotoxicity-induced neurodegeneration and found that CP-31398, a known compound that can rescue the structure and function of the tumor suppressor protein p53 mutant form and stabilize the active conformation of the p53 wild-type form, suppresses excitotoxicity-induced axon degeneration and cell body death. Moreover, CP-31398 suppresses mitochondrial dysfunction which has a strong correlation with excitotoxicity. Thus, our findings identify a compound that can serve as a novel modulator of neurodegeneration induced by glutamate excitotoxicity.
神经退行性变导致神经元功能障碍和退化,其触发因素包括遗传缺陷、自由基、损伤和谷氨酸兴奋性毒性等。其中,谷氨酸兴奋性毒性与包括 AD 和 ALS 在内的慢性疾病以及包括创伤性脑损伤在内的中枢神经系统急性损伤有关。神经退行性疾病表现出标志性的形态异常,如轴突退化和细胞体死亡。兴奋性毒性诱导的神经退行性变的分子机制很复杂,从一个角度解析一个分子机制有助于理解这个过程,但由于存在其他机制,仍然难以开发抑制兴奋性毒性诱导的退行性变的策略。因此,直接鉴定可以调节兴奋性毒性诱导的神经退行性变的化合物,并随后阐明其分子机制,是开发抑制神经退行性变的有效策略的有效方法。我们搜索了可以抑制兴奋性毒性诱导的神经退行性变的化合物,发现 CP-31398 是一种已知的化合物,可以挽救肿瘤抑制蛋白 p53 突变型的结构和功能,并稳定 p53 野生型的活性构象,可抑制兴奋性毒性诱导的轴突退化和细胞体死亡。此外,CP-31398 还可以抑制与兴奋性毒性密切相关的线粒体功能障碍。因此,我们的研究结果确定了一种可以作为谷氨酸兴奋性毒性诱导的神经退行性变的新型调节剂的化合物。