Recent advance in role of valosin-containing protein in pathogenesis of neurodegenerative diseases
10.3760/cma.j.cn115354-20250603-00318
- VernacularTitle:含缬氨酸蛋白在神经退行性疾病发病机制中的作用研究进展
- Author:
Jinping LI
1
;
Xing GUO
;
Hao YAO
Author Information
1. 南京医科大学第二附属医院麻醉科，南京 210011
- Publication Type:Journal Article
- Keywords:
Valosin-containing protein;
Neurodegenerative disease;
Protein homeostasis;
Ubiquitin-proteasome system;
Autophagy-lysosome pathway;
Pathological protein
- From:
Chinese Journal of Neuromedicine
2025;24(11):1174-1181
- CountryChina
- Language:Chinese
-
Abstract:
The core pathological hallmark of neurodegenerative diseases (NDDs) is the aberrant aggregation and impaired clearance of pathological proteins. Valosin-containing protein (VCP) plays a pivotal role in maintaining intracellular protein homeostasis by regulating the ubiquitin-proteasome system and autophagy-lysosome pathway. Pathogenic mutations or dysfunction of VCP can trigger widespread protein clearance defects, leading to pathological protein aggregation and neuronal death, and consequently contributing to NDDs progression. This article reviews the molecular structure and functions of VCP, as well as the role of VCP mutations or dysfunction in pathogenesis of inclusion body myopathy with Paget's disease of bone and frontotemporal dementia, Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis and Huntington's disease, so as to provide new strategies and directions for NDDs treatment.
