Department of Population Medicine and Diagnostic Sciences, College of Veterinary Medicine, Cornell University, S1 070 Schurman Hall, Ithaca, NY 14853, USA.
Vet Res Commun. 2012 Dec;36(4):221-6. doi: 10.1007/s11259-012-9533-y. Epub 2012 Aug 24.
Equine motor neuron disease (EMND) is a neurodegenerative disorder of unknown etiology affecting horses worldwide. Trans-Active Response DNA Binding Protein of 43 kDa (TDP-43) has been reported in the central nervous system (CNS) of several neurodegenerative conditions in humans including Amyotrophic Lateral Sclerosis (ALS) and assumed to play role in the disease. We examined whether horses afflicted with EMND express the TDP-43 in CNS. Ten horses with EMND and 6 controls of different ages and breed we enrolled. Detection of presence of TDP-43 protein in the CNS was analyzed by immunohistochemical staining using rabbit anti-human TARDBP (TDP-43) polyclonal antibody. Formalin fixed neuronal tissues from medulla, cervical, and lumbar spinal cord were harvested from EMND and from control horses. Sections were assigned randomly to TDP-43 treated or rabbit anti-IgG as control. Nuclear staining of TDP-43 was detected in one of the neural tissues of 75 % of EMND-positive and 0 of 0 % of control horses in the central nervous system (medulla, and/or cervical spinal cord and/or lumbar spinal cord). TDP-43 antibody was detected in the nucleus of EMND horses and no cytoplasmic staining was noted. As in ALS, there was no pattern of age clustering associated with the detection of TDP-43. This is the first report on the staining of TDP-43 in neuronal tissues of horses and suggests that TDP-43 may play a role in the pathogenesis of EMND. Further studies are needed to elucidate the etiologic role of this protein in the diseases.
马运动神经元病（EMND）是一种病因不明的神经退行性疾病，影响全球的马匹。转活化反应 DNA 结合蛋白 43kDa（TDP-43）已在人类几种神经退行性疾病的中枢神经系统（CNS）中被报道，包括肌萎缩侧索硬化症（ALS），并被认为在疾病中发挥作用。我们检查了是否患有 EMND 的马在中枢神经系统中表达 TDP-43。我们招募了 10 匹患有 EMND 的马和 6 匹不同年龄和品种的对照马。使用兔抗人 TARDBP（TDP-43）多克隆抗体通过免疫组织化学染色分析中枢神经系统中 TDP-43 蛋白的存在。从 EMND 和对照马的延髓、颈和腰脊髓中采集福尔马林固定的神经元组织。将切片随机分配给 TDP-43 处理或兔抗 IgG 作为对照。在中枢神经系统（延髓和/或颈脊髓和/或腰脊髓）中，在 75%的 EMND 阳性马的 1 种神经组织中检测到 TDP-43 的核染色，而在 0 例对照马中未检测到。在 EMND 马的细胞核中检测到 TDP-43 抗体，未观察到细胞质染色。与 TDP-43 的检测一样，在 ALS 中没有与年龄聚类相关的模式。这是首次报道 TDP-43 在马的神经元组织中的染色，表明 TDP-43 可能在 EMND 的发病机制中起作用。需要进一步的研究来阐明该蛋白在疾病中的病因作用。
