Sánchez-Elexpuru Gentzane, Serratosa José M, Sánchez Marina P
Laboratory of Neurology, IIS-Jiménez Díaz Foundation, UAM, Madrid, Spain.
Biomedical Research Network Center on Rare Diseases (CIBERER), Madrid, Spain.
Epilepsia. 2017 Mar;58(3):467-475. doi: 10.1111/epi.13656. Epub 2017 Jan 18.
To search for new therapies aimed at ameliorating the neurologic symptoms and epilepsy developing in patients with Lafora disease.
Lafora disease is caused by loss-of-function mutations in either the EPM2A or EPM2B genes. Epm2a and Epm2b mice display neurologic and behavioral abnormalities similar to those found in patients. Selenium is a potent antioxidant and its deficiency has been related to the development of certain diseases, including epilepsy. In this study, we investigated whether sodium selenate treatment improved the neurologic alterations and the hyperexcitability present in the Epm2b mouse model.
Sodium selenate ameliorates some of the motor and memory deficits and the sensitivity observed with pentylenetetrazol (PTZ) treatments in Epm2b mice. Neuronal degeneration and gliosis were also diminished after sodium selenate treatment.
Sodium selenate could be beneficial for ameliorating some symptoms that present in patients with Lafora disease.
寻找旨在改善拉福拉病患者出现的神经症状和癫痫的新疗法。
拉福拉病由EPM2A或EPM2B基因的功能丧失突变引起。Epm2a和Epm2b小鼠表现出与患者相似的神经和行为异常。硒是一种强效抗氧化剂，其缺乏与包括癫痫在内的某些疾病的发生有关。在本研究中，我们调查了硒酸钠治疗是否能改善Epm2b小鼠模型中存在的神经改变和过度兴奋性。
硒酸钠改善了Epm2b小鼠在接受戊四氮（PTZ）治疗时观察到的一些运动和记忆缺陷以及敏感性。硒酸钠治疗后神经元变性和胶质增生也有所减轻。
硒酸钠可能有助于改善拉福拉病患者出现的一些症状。
