含有瓦洛辛的蛋白质 (VCP):对其多种分子功能和临床表型的综述
Carly S Pontifex1, Mashiat Zaman1,2,3, Roberto D Fanganiello4
1Hotchkiss Brain Institute, Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada.
International journal of molecular sciences
|June 19, 2024
概括
含有瓦洛辛的蛋白质 (VCP/p97) 对于细胞功能至关重要,其突变会导致多系统蛋白质病变,影响各种组织. 了解风险投资方针的理解
科学领域:
- 细胞生物学 细胞生物学
- 分子生物学分子生物学
- 遗传学 遗传学 是一个
背景情况:
- 含有瓦洛的蛋白质 (VCP/p97) 是一个AAA-ATPase,参与各种细胞过程.
- VCP/p97在蛋白质稳态,DNA修复和线粒分裂中起着至关重要的作用.
- 功能障碍的VCP/p97导致多系统蛋白质病变.
研究的目的:
- 审查VCP/p97.7.的分子功能.
- 为了检查VCP/p97.7.的突变格局.
- 为了阐明VCP疾病的表型表现.
主要方法:
- 对VCP/p97功能的文献综述.
- 对VCP/p97突变和相关疾病的分析.
- 综合有关VCP/p97相关病理学的当前知识.
主要成果:
- VCP/p97对于蛋白质质量控制,ERAD,自和DNA损伤反应至关重要.
- VCP突变导致多系统蛋白质病变,影响骨肌肉,大脑和神经元.
- 与VCP相关的疾病的疾病进展是不可预测的.
结论:
- VCP/p97是众多细胞通路的中心枢纽.
- VCP突变强调了蛋白质稳态与系统性疾病之间的联系.
- 需要进一步的研究来预测和管理VCP疾病的进展.
相关概念视频
Protein Complexes with Interchangeable Parts
2.5K
Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order...
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order...
2.5K
COP Coated Vesicles
7.8K
Membrane-enclosed structures called vesicles transport proteins and lipids across the cell. The vesicles derive their cargo from the plasma membrane, Golgi, ER, or endosome. Coated vesicles are spherical, protein-coated carriers with a 50–100 nm diameter that mediate bidirectional transport between the ER and the Golgi. The distribution of proteins between the ER and Golgi complex is dynamic and is maintained by different coated vesicles. Their formation is driven by the assembly of...
7.8K
Overview of Secretory Vesicles
8.5K
Secretory vesicles, also known as dense core vesicles (DCVs), are membrane-bound vesicles that transport secretory proteins, such as hormones or neurotransmitters. Regulated secretory vesicles transport proteins from the trans-Golgi network to the exterior of the cell. Proteins present in regulated secretory vesicles are required to be rapidly exocytosed in large amounts upon a specific stimulus.
Various proteins regulate the aggregation of molecules inside the secretory vesicles. Chromogranins...
Various proteins regulate the aggregation of molecules inside the secretory vesicles. Chromogranins...
8.5K
Vesicular Tubular Clusters
2.5K
After budding out from the ER membrane, some COPII vesicles lose their coat and fuse with one another to form larger vesicles and interconnected tubules called vesicular tubular clusters or VTCs. These clusters constitute a compartment at the ER-Golgi interface known as ERGIC (Endoplasmic Reticulum Golgi Intermediate Compartment). The ERGIC is a mobile membrane-bound cargo transport system that sorts proteins secreted from ER and delivers them to the Golgi.
With the help of motor proteins such...
With the help of motor proteins such...
2.5K
Protein Complex Assembly
10.6K
Proteins can form homomeric complexes with another unit of the same protein or heteromeric complexes with different types. Most protein complexes self-assemble spontaneously via ordered pathways, while some proteins need assembly factors that guide their proper assembly. Despite the crowded intracellular environment, proteins usually interact with their correct partners and form functional complexes.
Many viruses self-assemble into a fully functional unit using the infected host cell to...
Many viruses self-assemble into a fully functional unit using the infected host cell to...
10.6K
Covalently Linked Protein Regulators
6.8K
Proteins can undergo many types of post-translational modifications, often in response to changes in their environment. These modifications play an important role in the function and stability of these proteins. Covalently linked molecules include functional groups, such as methyl, acetyl, and phosphate groups, and also small proteins, such as ubiquitin. There are around 200 different types of covalent regulators that have been identified.
These groups modify specific amino acids in a protein....
These groups modify specific amino acids in a protein....
6.8K


