两个FAM134B异型在肌体发生过程中差异调节ER动态
Viviana Buonomo1,2, Kateryna Lohachova3,4, Alessio Reggio1,5
1Telethon Institute of Genetics and Medicine (TIGEM), 80078, Pozzuoli, Italy.
The EMBO journal
|January 6, 2025
概括
这项研究揭示了FAM134B异型是如何调节肌肉细胞分化过程中的内等质网膜 (ER) 重塑的. 向FAM134B2的转变通过ER-phagy驱动ER重组,保持肌肉细胞平衡.
科学领域:
- 细胞生物学 细胞生物学
- 分子生物学分子生物学
- 肌肉生理学 肌肉生理学
背景情况:
- 细胞内膜网膜 (ER) 的可塑性和ER-phagy对于细胞平衡至关重要.
- FAM134B是一种ER-phagy受体,参与ER重塑.
研究的目的:
- 研究FAM134B异型体在肌肉形成过程中ER重塑中的作用.
- 阐明FAM134B异型和ER-phagy在分化髓细胞中的相互作用.
主要方法:
- 在肌肉发育过程中研究了FAM134B异形动力学.
- 在肌管中利用了淘汰和再表达模型.
- 分析了ER形态和蛋白质组景观.
主要成果:
- 在肌肉发育过程中,法典FAM134B1被降解,而FAM134B2被上调.
- 通过ER-phagy,FAM134B2促进了ER的重塑和重组.
- 在FAM134B2中,FAM134B2的再表达能够挽救FAM134B淘汰神经管中的异常蛋白质组和ER扩张.
结论:
- 精细调整FAM134B异构体,可以在肌体发生过程中调节ER动态.
- 在肌肉细胞中,FAM134B2在ER恒温中起着关键作用.
- 了解这些机制为肌肉细胞生物学提供了洞察力.
相关概念视频
Master Transcription Regulators
6.9K
Master transcription regulators are regulatory proteins that are predominantly responsible for regulating the expression of multiple genes. Often these genes work in concert to drive a complex process. Activation of a master transcription regulator can lead to a cascade of transcriptional activation necessary for that outcome. These regulators can directly bind to the regulatory sequences of the various genes involved, or they can indirectly regulate transcription by binding to regulatory...
6.9K
Regulation of the Unfolded Protein Response
2.4K
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...
2.4K
Directing Proteins to the Rough Endoplasmic Reticulum
7.1K
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...
7.1K
The Unfolded Protein Response
4.4K
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...
4.4K
Protein Modifications in the RER
5.0K
Modification of secretory and transmembrane proteins entering the rough ER begins in the ER lumen. These modifications aid in protein folding and stabilize the acquired tertiary structure. Protein modifications in the rough ER co-occur at different stages of protein folding.
Broadly, these modifications can be categorized into four main categories — glycosylation, formation of disulfide bonds, assembly of protein subunits, and specific proteolytic cleavages like removal of signal...
Broadly, these modifications can be categorized into four main categories — glycosylation, formation of disulfide bonds, assembly of protein subunits, and specific proteolytic cleavages like removal of signal...
5.0K
Assembly of the Lipid Bilayer in the ER
3.1K
Biological membranes are more than just a barrier separating cell cytoplasm from the outside environment. They are highly dynamic and help maintain the integrity and physiological stability of the cells as well as membrane-bound organelles. Membranes also play vital roles in cell-to-cell and intracellular communication.
A large chunk of any biological membrane is composed of phospholipids. These lipids have a heterogeneous distribution across different subcellular organelles and even between...
A large chunk of any biological membrane is composed of phospholipids. These lipids have a heterogeneous distribution across different subcellular organelles and even between...
3.1K


