在肌缩侧面硬化症中神经回路功能障碍
Andrea Salzinger1,2, Vidya Ramesh1,2, Shreya Das Sharma1,2
1UK Dementia Research Institute, University of Edinburgh, Edinburgh EH16 4SB, UK.
Cells
|May 24, 2024
概括
肌缩侧面硬化症 (ALS) 损害皮质脊髓运动回路,影响上部运动神经元 (UMN) 和下部运动神经元 (LMN). 研究探讨了运动电路功能障碍的机制和使用干细胞模型的潜在治疗方法.
科学领域:
- 神经科学是一个神经科学.
- 神经学 神经学
- 细胞生物学 细胞生物学
背景情况:
- 肌缩侧面硬化 (ALS) 是一种神经退行性疾病,主要影响皮质脊髓运动回路.
- 这种疾病涉及上部运动神经元 (UMN) 和下部运动神经元 (LMN) 的损失,导致肌肉缩和运动障碍.
- 尽管进行了广泛的研究,目前对ALS的疾病修饰治疗仍然有限.
研究的目的:
- 审查当前关于ALS皮质运动电路功能障碍机制的研究.
- 总结关于UMN和LMN功能障碍,神经肌肉结变性和ALS中的质细胞作用的知识.
- 突出人类干细胞模型在研究ALS神经电路病理学方面的实用性.
主要方法:
- 对患者研究的审查.
- 对动物ALS模型的分析.
- 对用于ALS研究的人类干细胞模型的检查.
主要成果:
- 皮质运动电路功能障碍是ALS病理生理学的核心.
- 关键方面包括UMN退化,LMN过激,神经肌肉结缺陷和非细胞自主质贡献.
- 人类干细胞技术为模拟ALS中复杂的神经回路提供了先进的平台.
结论:
- 了解运动电路功能障碍对于开发ALS疗法至关重要.
- 人类干细胞模型为ALS机制提供了宝贵的见解.
- 使用这些模型进行进一步的研究可以加速开发有效的ALS治疗方法.
相关概念视频
Neural Regulation
39.4K
Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
39.4K
Parkinson's Disease: Overview
528
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
528
Cross-bridge Cycle
117.3K
As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
117.3K
Chemical Synapses
8.8K
Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
8.8K
Disorders of the Nervous Tissue
1.2K
Nervous tissue is a vital component of the human body's communication system, enabling us to perceive and respond to stimuli. However, like all other tissues, it is vulnerable to disorders and diseases that can significantly impact our neurological functioning.
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
1.2K
Lysosomal Hydrolases
3.8K
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
3.8K


