Section of Developmental Physiology and Pathology, Faculty of Education, Okayama University, Okayama, Japan.
Department of Child Neurology, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama University, Okayama, Japan.
Epilepsia. 2022 Jul;63(7):e80-e85. doi: 10.1111/epi.17295. Epub 2022 May 28.
Thioredoxin, encoded by Txn1, is a critical antioxidant that protects against oxidative damage by regulating the dithiol/disulfide balance of interacting proteins. We recently discovered the Adem rat, an epileptic rat harboring the Txn1-F54L mutation, characterized by wild running and vacuolar degeneration in the midbrain. This study aimed to characterize the classification of epilepsy in Adem rats. We performed simultaneous video-electroencephalographic recordings, magnetic resonance imaging, neurotransmitter measurements using gas chromatography-mass spectrometry (GC-MS), and immunohistochemistry. Adem rats exhibited absence, tonic, and focal seizures. The type of epilepsy was classified as combined generalized and focal epilepsy. Neurotransmitters in the midbrain and cortex were measured at 3 weeks of age, when neuronal cell death occurs in the midbrain. The results of GC-MS ruled out the dominance of the excitatory system in the midbrain and cortex of Adem rats. Activation of astrocytes and microglia was more pronounced at 5 weeks of age, at which time epileptic seizures occurred frequently. The underlying pathology in Adem rats remains unknown. However, glial cell activation and inflammation may play a significant role in the occurrence of epilepsy.
硫氧还蛋白由 Txn1 编码，是一种关键的抗氧化剂，通过调节相互作用蛋白的二硫键/巯基平衡来防止氧化损伤。我们最近发现了 Adem 大鼠，这是一种携带 Txn1-F54L 突变的癫痫大鼠，其特征是野生奔跑和中脑空泡变性。本研究旨在对 Adem 大鼠的癫痫分类进行特征描述。我们进行了同步视频脑电图记录、磁共振成像、使用气相色谱-质谱法 (GC-MS) 测量神经递质以及免疫组织化学。Adem 大鼠表现出失神、强直和局灶性发作。癫痫的类型被归类为全面性和局灶性癫痫合并。在神经元细胞死亡发生于中脑的 3 周龄时，测量了中脑和皮质中的神经递质。GC-MS 的结果排除了 Adem 大鼠中脑和皮质中兴奋性系统的主导地位。星形胶质细胞和小胶质细胞的激活在 5 周龄时更为明显，此时癫痫发作频繁。Adem 大鼠的潜在病理仍不清楚。然而，神经胶质细胞的激活和炎症可能在癫痫的发生中起重要作用。
