内质网膜自受体TEX264驱动表皮分化,并且在达利尔病中失调
bioRxiv : the preprint server for biology
|September 4, 2025
概括
TEX264通过选择性自过程促进皮肤屏障形成,从而推动皮肤分化. 这一发现揭示了诸如达里尔病之类的角化疾病.
科学领域:
- 细胞生物学
- 皮肤病学
- 自研究
背景情况:
- 皮质细胞的分化形成了表皮屏障,这是通过一种鲜为人知的代谢过程.
- 在角质细胞角化过程中,有机细胞和核分解是关键事件.
研究的目的:
- 阐明在角质细胞分化过程中有机细胞降解的分子机制.
- 研究TEX264在角化和表皮屏障形成过程中的作用.
主要方法:
- 人类器官类型表皮的现场共焦成像.
- 在ER应激和分化角质细胞中分析TEX264表达.
- 对TEX264的基因淘汰和功能突变研究.
- 检查患者的活检和达里尔病的器官模型.
主要成果:
- 在角化过程中观察到内等质网膜 (ER) 碎片化和溶酶体吞.
- 在角化和ER压力过程中,TEX264是一种网膜受体.
- TEX264促进了皮质细胞的分裂和消除,以及皮质细胞的核收缩.
- 在达里尔病中,TEX264 缺乏破坏了表皮成熟,其水平增加了.
结论:
- TEX264是角质细胞分化和角化的一个关键调节剂.
- 在表皮屏障形成过程中,由TEX264介导的网膜食对于有序的器官清除至关重要.
- TEX264的失调与ER功能障碍相关的遗传角化疾病有关.
相关概念视频
Receptor Downregulation in MVBs
2.1K
Multivesicular bodies (MVBs) are mature endosomes that sort ubiquitinated proteins and then fuse with lysosomes to degrade the sorted proteins. Epidermal growth factor (EGF) and its receptor (EGFR) form a complex that can be internalized through endocytosis, sorted into an MVB, and later degraded.
The EGFR can initiate signaling pathways that lead to cell proliferation, migration, and differentiation. Overexpression of EGFR stimulates cells to proliferate. Excessive EGFR...
The EGFR can initiate signaling pathways that lead to cell proliferation, migration, and differentiation. Overexpression of EGFR stimulates cells to proliferate. Excessive EGFR...
2.1K
ER Retrieval Pathway
3.9K
In the secretory pathway, vesicles transport proteins from one cellular compartment to another in forward transport to deliver the protein to its correct location. Occasionally, misfolded proteins and incorrect proteins escape their original compartments, and a retrieval pathway is used to return the escaped proteins to their original compartment.
The ER uses many checkpoints to prevent the entry of incorrectly folded or a resident protein as cargo onto a transport vesicle. These mechanisms...
The ER uses many checkpoints to prevent the entry of incorrectly folded or a resident protein as cargo onto a transport vesicle. These mechanisms...
3.9K
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
Post-translational Translocation of Proteins to the RER
5.9K
A sizable fraction of proteins destined for ER are first synthesized in the cell cytosol and then transported across the ER membrane–a process called post-translational translocation. Similar to cotranslationally translocated proteins, these proteins also use the Sec translocon complex to enter the ER lumen.
Targeting proteins to the ER
Hsp40 and Hsp70 chaperone molecules bind the translated proteins in the cytosol to prevent their folding. The chaperone binding helps to keep the signal...
Targeting proteins to the ER
Hsp40 and Hsp70 chaperone molecules bind the translated proteins in the cytosol to prevent their folding. The chaperone binding helps to keep the signal...
5.9K
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


