在神经发育障碍中,内质网膜蛋白质稳定性的障碍
Danilo B Medinas1, Nayrob Pereira1,2, Rodolfo Pereira1,2
1Department of Biochemistry, Institute of Chemistry, University of Sao Paulo, Sao Paulo, Brazil.
Biochemical Society transactions
|July 28, 2025
概括
细胞内膜网膜 (ER) 蛋白质平衡的破坏与神经发育障碍有关. 了解ER蛋白质稳定机制对于解决由蛋白质生物生成缺陷引起的神经系统疾病至关重要.
科学领域:
- 细胞生物学 细胞生物学
- 神经科学是一个神经科学.
- 遗传学 是一个遗传学.
背景情况:
- 细胞内膜网膜 (ER) 对于合成膜和分泌蛋白质至关重要.
- ER蛋白质稳定通过复杂的细胞机制维持蛋白质稳定.
- 神经发育过程严重依赖蛋白质生物发生和分泌途径.
研究的目的:
- 审查ER蛋白质稳定在神经发育中的作用.
- 要突出ER蛋白生物生成中的缺陷如何导致神经系统疾病.
- 确定ER蛋白质稳定和神经发育领域的关键研究问题.
主要方法:
- 文献审查和证据汇编.
- 对影响ER蛋白生物发生的遗传缺陷的分析.
- 对ER蛋白质稳定和神经发育结果的当前知识的综合.
主要成果:
- 在ER蛋白质稳定节点的遗传缺陷可以严重损害神经发育.
- 在ER蛋白生物生成步骤中出现的干扰与神经系统疾病有关.
- 神经发育期间广泛的蛋白质组重塑挑战了ER蛋白质稳定.
结论:
- 对于正常的神经发育来说,ER蛋白质稳定是至关重要的.
- 功能障碍的ER蛋白生物发生是神经疾病的重要贡献者.
- 需要进一步的研究来阐明神经系统疾病中ER蛋白质稳定机制.
更多相关视频
07:43Immunohistochemical Visualization of Hippocampal Neuron Activity After Spatial Learning in a Mouse Model of Neurodevelopmental Disorders
Published on: May 12, 2015
11.4K
10:50Molecular Modulation by Lentivirus-Delivered Specific shRNAs in Endoplasmic Reticulum Stressed Neurons
Published on: April 24, 2021
1.1K
相关概念视频
Smooth Endoplasmic Reticulum
6.2K
Smooth endoplasmic reticulum or smooth ER is a sub-organelle with specialized functions in animal cells and plant cells. It is often associated with the tubule morphology of the endoplasmic reticulum.
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...
6.2K
Export of Misfolded Proteins out of the ER
3.9K
After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
3.9K
The Unfolded Protein Response
5.1K
The ER is the hub of protein synthesis in a cell. It has robust systems to quality control protein folding and also for degradation of terminally misfolded proteins. Under normal conditions, a small proportion of misfolded proteins that cannot be salvaged need to be transported to the cytoplasm by the ER-associated degradation or ERAD pathways. However, if the ERAD cannot handle the misfolded proteins, the cell activates the unfolded protein response or UPR to adjust the protein folding...
5.1K
Role of ER in the Secretory Pathway
5.6K
Eukaryotic cells have a special pathway that enables communication between various intracellular membrane-bound compartments and also with the extracellular environment. This pathway is termed as the secretory pathway.
Components of the secretory pathway
About a third of proteins synthesized in the cell are sorted via the secretory route. They shuffle between different compartments in membrane-bound vesicles until they reach their final destination. The main intracellular compartments involved...
Components of the secretory pathway
About a third of proteins synthesized in the cell are sorted via the secretory route. They shuffle between different compartments in membrane-bound vesicles until they reach their final destination. The main intracellular compartments involved...
5.6K
Protein Folding Quality Check in the RER
3.8K
ER is the primary site for the maturation and folding of soluble and transmembrane secretory proteins. The calnexin cycle is a specific chaperone system that folds and assesses the confirmation of N-glycosylated proteins before they can exit the ER lumen. The primary players of this quality check pipeline are the lectins, ER-resident chaperones, and a glucosyl transferase enzyme. In case the calnexin system in the lumen fails to salvage a misfolded protein, it is transported to the cytoplasm...
3.8K
Disorders of the Nervous Tissue
1.6K
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...
1.6K
