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相关概念视频

Regulation of the Unfolded Protein Response01:31

Regulation of the Unfolded Protein Response

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Inositol-requiring kinase one or IRE1 is the most conserved eukaryotic unfolded protein response (UPR) receptor. It is a type I transmembrane protein kinase receptor with a distinctive site-specific RNase activity. As the binding mechanics of the misfolded proteins with the N-terminal domain of IRE-1 are unclear, three binding models — direct, indirect, and allosteric -- are proposed for receptor activation. Nevertheless, it is known that once a misfolded protein associates with IRE1, it...
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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...
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The Unfolded Protein Response01:37

The Unfolded Protein Response

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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...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin01:26

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

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Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
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Directing Proteins to the Rough Endoplasmic Reticulum01:34

Directing Proteins to the Rough Endoplasmic Reticulum

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The organelle-specific signaling sequences direct proteins synthesized in the cytosol to their final destination like ER, mitochondria, peroxisomes, etc. Some of the proteins directed to ER are then trafficked via vesicles to other organelles within the cell or the extracellular environment through the Golgi complex. For example, the rough ER synthesizes soluble proteins for transportation to the lysosomes or secretion out of the cell. It can also synthesize transmembrane proteins that can...
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Functional Characterization of Endogenously Expressed Human RYR1 Variants
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瑞诺丁受体1型受体含量下降诱导的内质网膜压力是肌肉病变的标志.

Jeremy Vidal1, Eric A Fernandez2, Martin Wohlwend3

  • 1Institute of Sport Sciences and Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland.

Journal of cachexia, sarcopenia and muscle
|November 15, 2023
PubMed
概括
此摘要是机器生成的。

降低的氨酸受体1型 (RyR1) 蛋白在肌肉病中很常见,导致细胞内膜网膜 (ER) 应激和肌肉功能障碍. 这项研究揭示了RyR1枯竭是各种肌肉病的关键因素,有助于疾病的发病.

关键词:
在CHOP中使用CHOP.在GRP78-Bipip中使用.和是最重要的.脂肪液滴滴滴滴滴滴滴滴滴滴滴滴滴滴滴滴线粒细胞衰变 (mitophagy) 是一种神经衰变的过程.肌肉 肌肉 肌肉 肌肉 肌肉

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Genetic and Biochemical Approaches for In Vivo and In Vitro Assessment of Protein Oligomerization: The Ryanodine Receptor Case Study
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相关实验视频

Last Updated: Jul 11, 2025

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Genetic and Biochemical Approaches for In Vivo and In Vitro Assessment of Protein Oligomerization: The Ryanodine Receptor Case Study
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科学领域:

  • 肌肉生理学和分子生物学
  • 细胞应激反应细胞应激反应
  • 神经肌肉疾病机制的神经肌肉疾病机制

背景情况:

  • 降低氨酸受体1型 (RyR1) 蛋白质是衰退型RYR1相关肌病的标志.
  • 在其他肌肉病症中,RyR1减少的作用及其潜在机制仍然不太清楚.

研究的目的:

  • 在RYR1相关疾病之外,研究各种肌肉病中的RyR1蛋白水平.
  • 阐明减少RyR1蛋白质对肌肉病理有所贡献的分子机制.

主要方法:

  • 对公开可用的数据集和来自炎症和线粒体肌肉病的人类肌肉样本的分析.
  • 利用一种可诱导的肌肉特异性RYR1衰退的小鼠模型和C2C12肌肉细胞中的RyR1敲击.
  • 采用蛋白质组学,脂组学,分子生物学和电子显微镜来评估RyR1减少效应.

主要成果:

  • 减少的RYR1转录和蛋白质水平被观察到在死性肌肉病,包容性身体肌肉病,多聚炎,皮肤肌肉病,肌肉性缩症,杜恩,贝克尔和四肢腰带肌肉缩症.
  • 在体外和体内模型中,RyR1的耗尽导致了ER-线粒体接触的减少,Ca2+转移的受损,线粒体功能障碍和有害脂的积累.
  • 在RyR1-缺陷肌肉中,持续地发现了内质网膜 (ER) 应激标志物 (GRP78-Bip) 和亲位蛋白 (CHOP-DDIT3) 的增加.

结论:

  • 降低 RyR1 蛋白质是各种肌肉病的共同特征,不仅限于与 RYR1 相关的疾病.
  • 通过诱导ER压力,RyR1枯竭显著促进肌病病原性.
  • 这些发现突出了ER压力作为关键机制,将RyR1缺乏与肌肉疾病联系起来.