杏仁体TDP-43病理与行为功能障碍和费里丁积累有关,在肌缩性侧面硬化症中
Olivia M Rifai1,2, Fergal M Waldron3, Judi O'Shaughnessy4
1Centre for Discovery Brain Sciences, University of Edinburgh, UK.
bioRxiv : the preprint server for biology
|June 10, 2024
概括
杏仁体中的病态TDP-43积累与零星ALS (sALS) 的行为变化相关. 大脑铁 (费里丁) 的增加可能会在这些临床症状之前出现,这表明潜在的早期生物标志物.
科学领域:
- 神经科学是一个神经科学.
- 神经病理学神经病理学
- 神经退行性疾病 神经退行性疾病
背景情况:
- 肌缩性侧面硬化和前光谱障碍 (ALS-FTD) 的认知和行为症状与TDP-43蛋白积累有关.
- 了解特定的大脑区域和TDP-43的病理形式对于诊断和治疗ALS-FTD至关重要.
研究的目的:
- 研究特定脑区TDP-43病理与零星ALS (sALS) 的认知/行为症状之间的关系.
- 为了确定潜在的生物标志物用于sALS.早期疾病检测.
主要方法:
- 在30名sALS患者中检查了来自6个区域的死后脑组织.
- 使用爱丁堡认知ALS屏幕 (ECAS) 进行行为评估.
- 分析了化TDP-43 (pTDP-43) 和TDP-43合体 (TDP-43^APT) 病理学,包括费里水平.
主要成果:
- 在ECAS行为屏幕准确预测pTDP-43积累在行为相关的大脑区域 (100%的特异性,86%的灵敏度).
- 杏仁体病理是sALS行为功能障碍的最强相关因子.
- 杏仁体中神经内pTDP-43和TDP-43^APT病理与行为症状和增加的费里水平相关.
结论:
- 杏仁体内的神经内pTDP-43和TDP-43^APT病理与sALS的行为症状有关.
- 增加的费里水平,可能与早期的TDP-43病理有关,可能作为早期的,区域特定的ALS成像生物标志物.
相关概念视频
03:03Amyloid Fibrils
9.5K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
9.5K
01:26Alzheimer's Disease: Overview
462
Alzheimer's Disease (AD) is a continually advancing neurodegenerative disorder, distinguished by escalating memory loss, cognitive dysfunction, and dementia. The disease unfolds in three stages: preclinical, mild cognitive impairment (MCI), and dementia. Its onset is insidious, and the progression gradual, with the cause not well explained by other disorders.
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ...
462
01:22Alzheimer's Disease: Treatment
179
Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
179
01:37Neural 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
01:15Parkinson's Disease: Overview
527
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...
527
01:22Lysosomal 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