Mesnard Nichole A, Haulcomb Melissa M, Tanzer Lisa, Sanders Virginia M, Jones Kathryn J
Department of Microbiology and Immunology, Loyola University Medical Center, Maywood, Illinois ; Research and Development Service, Hines VA Hospital, Hines, Illinois ; Department of Anatomy and Cell Biology, Indiana University, Indianapolis, Indiana.
Research and Development Service, Hines VA Hospital, Hines, Illinois ; Department of Anatomy and Cell Biology, Indiana University, Indianapolis, Indiana.
J Neurodegener Regen. 2013 Fall;4(1):21-25.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving progressive loss of motoneurons (MN). Axonal pathology and presynaptic deaf-ferentation precede MN degeneration during disease progression in patients and the ALS mouse model (mSOD1). Previously, we determined that a functional adaptive immune response is required for complete functional recovery following a facial nerve crush axotomy in wild-type (WT) mice. In this study, we investigated the effects of facial nerve crush axotomy on functional recovery and facial MN survival in presymptomatic mSOD1 mice, relative to WT mice. The results indicate that functional recovery and facial MN survival levels are significantly reduced in presymptomatic mSOD1, relative to WT, and similar to what has previously been observed in immunodeficient mice. It is concluded that a potential immune system defect exists in the mSOD1 mouse that negatively impacts neuronal survival and regeneration following target disconnection associated with peripheral nerve axotomy.
肌萎缩侧索硬化症（ALS）是一种致命的神经退行性疾病，涉及运动神经元（MN）的渐进性丧失。在患者和ALS小鼠模型（mSOD1）的疾病进展过程中，轴突病理学和突触前去传入先于MN变性。此前，我们确定野生型（WT）小鼠面神经挤压轴突切断术后的完全功能恢复需要功能性适应性免疫反应。在本研究中，我们研究了面神经挤压轴突切断术对症状前mSOD1小鼠相对于WT小鼠的功能恢复和面部MN存活的影响。结果表明，相对于WT，症状前mSOD1小鼠的功能恢复和面部MN存活水平显著降低，与先前在免疫缺陷小鼠中观察到的情况相似。得出的结论是，mSOD1小鼠存在潜在的免疫系统缺陷，这对与周围神经轴突切断术相关的靶标断开后的神经元存活和再生产生负面影响。
