LYVAC/PDZD8是一种溶酶体真空仪
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在PubMed上查看摘要
概括
此摘要是机器生成的。溶解体真空化是一种常见的疾病,由ER固蛋白PDD8 (转名为LYVAC) 介导. LYVAC感知了脂质,驱动了ER-to-lysosome转移和透扩张,揭示了一个关键的真空化机制.
科学领域
- 细胞生物学
- 分子机制
- 病理生理学
背景情况
- 在各种疾病中观察到溶酶体真空化,但其潜在的分子机制尚不清楚.
- 涉及诱导溶酶体真空化的特定蛋白质和途径需要进一步阐明.
研究的目的
- 确定溶解体真空化的分子媒介.
- 阐明内网 (ER) 定蛋白质对溶解体功能障碍的作用机制.
主要方法
- 使用人类细胞系来研究溶酶体真空化.
- 采用生物化学和成像技术研究蛋白质招募和脂质转移.
- 分析了PDZD8对各种真空诱导物的作用.
主要成果
- 已确定含有PDZ域8 (PDZD8),建议将其更名为 lysosomal vacuolator (LYVAC),作为 lysosomal vacuolation的一般媒介.
- 证明多种诱导剂在溶解体透应激上融合,通过多价值相互作用招募LYVAC.
- 展示了LYVAC的脂质转移域感知酸和胆固醇,介导ER-to-lysosome脂质流和lysosomal扩张.
结论
- 通过脂质转移和透应激,LYVAC是一种关键的ER定蛋白质.
- 这些发现揭示了溶酶体真空化的基本机制,具有显著的病理生理影响.
相关概念视频
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,...
Lysosomes are membrane-enclosed spherical sacs derived from the Golgi apparatus. The most important function of the lysosome is degrading macromolecules and biological polymers that are released during membrane trafficking events such as the secretory, endocytic, autophagic, and phagocytic pathways. The degradation is carried out by several hydrolytic enzymes active in an acidic environment of the lysosomal lumen. These acid hydrolases are involved in cellular processes such as cell signaling,...
Eukaryotic cells use different mechanisms to eliminate toxic waste obsolete and worn-out substances. Lysosomes play a pivotal role in this, and hence, these substances are carried to the lysosome from other parts of the cell and extracellular space through different pathways. The most elaborately studied pathways to the lysosome are the endocytic pathways.
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
Intraluminal vesicles (ILVs) are small vesicles 50-80 nm in diameter formed during the maturation of early endosomes. A specialized endosome containing numerous ILVs is called a multivesicular body (MVB). ILVs contain internalized molecules such as antigens, nucleic acids, proteins, and metabolites. Some of these molecules are released from the MVBs inside exosomes and are transported to other cells. Other MVBs contain molecules that are retained in the ILVs and are later degraded within the...
V-type pumps are ATP-driven pumps found in the vacuolar membranes of plants, yeast, endosomal and lysosomal membranes of animal cells, plasma membranes of a few specialized eukaryotic cells, and some prokaryotes. They are also known as the V1Vo-ATPase, that couple ATP hydrolysis to transport protons against a concentration gradient.
The peripheral or cytosolic V1 domain with eight subunits is involved in ATP hydrolysis. The integral or transmembrane V0 domain containing at least five subunits...
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...