Abstract：
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.
摘要：
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醇/二硫化物平衡来防止氧化损伤。我们最近发现了Adem老鼠,携带Txn1-F54L突变的癫痫大鼠，以中脑狂野奔跑和空泡变性为特征。本研究旨在表征Adem大鼠癫痫的分类。我们同时进行了视频脑电图记录，磁共振成像，使用气相色谱-质谱（GC-MS）进行神经递质测量，和免疫组织化学。Adem大鼠表现出缺失，补品,和局灶性癫痫发作。癫痫的类型分为全身性和局灶性癫痫。在3周龄时测量中脑和皮质的神经递质，当神经元细胞死亡发生在中脑。GC-MS的结果排除了Adem大鼠中脑和皮质中兴奋系统的优势。星形胶质细胞和小胶质细胞的激活在5周龄时更为明显，此时癫痫发作频繁发生。Adem大鼠的潜在病理学仍然未知。然而,神经胶质细胞活化和炎症反应可能在癫痫的发生中起重要作用。
