CHANGES IN NEUROMUSCULAR JUNCTIONS IN ALS

ALS 神经肌肉接头的变化

• 批准号: 8167050
• 负责人: JAMES B CARESS
• 金额: $ 0.17万
• 依托单位: WAKE FOREST UNIVERSITY HEALTH SCIENCES
• 依托单位国家: 美国
• 财政年份: 2010
• 资助国家: 美国
• 起止时间: 2010-03-01 至 2011-02-28
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8167050
• 关键词: Autopsy; Axon; Back; Cell Death; Cells; Cessation of life; Clinical; Computer Retrieval of Information on Scientific Projects Database; Disease; Funding; Grant; Human; Institution; Motor; Motor Neurons; Muscle Weakness; Muscle denervation procedure; Neuromuscular Junction; Neuropathy; Process; Research; Research Personnel; Resources; Secondary to; Source; Spinal Cord; Symptoms; United States National Institutes of Health; motor neuron degeneration; neuronal cell body

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. In ALS, the prominent clinical feature is weakness presumed to be secondary to degeneration of motor neurons. However, the death of these cells occurs late in the disease process and there are large numbers of surviving motor neuron cell bodies in human postmortem material that should be able to support considerable strength. Denervation of muscles or malfunction of the neuromuscular junction appears to occur prior to the onset of overt clinical symptoms and indeed is most likely the cause of muscle weakness in ALS as opposed to motor neuron death in the spinal cord. These results have prompted the hypothesis that weakness in ALS may be initiated by a dying back motor axon neuropathy which, in turn, may cause disruption in the cell bodies.

该子项目是利用该技术的众多研究子项目之一 资源由 NIH/NCRR 资助的中心拨款提供。子项目和 研究者 (PI) 可能已从 NIH 的另一个来源获得主要资金， 因此可以在其他 CRISP 条目中表示。列出的机构是 中心，不一定是研究者的机构。 在 ALS 中，突出的临床特征是推测继发于运动神经元变性的无力。然而，这些细胞的死亡发生在疾病过程的后期，并且在人类死后材料中存在大量幸存的运动神经元细胞体，它们应该能够支持相当大的强度。肌肉去神经支配或神经肌肉接头功能障碍似乎发生在明显的临床症状出现之前，并且实际上最有可能是 ALS 中肌肉无力的原因，而不是脊髓中运动神经元死亡。这些结果提出了这样的假设：ALS 的无力可能是由垂死的背部运动轴突神经病变引起的，而这种病变反过来又可能导致细胞体的破坏。