Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410008, China.
Department of Neurology, Multi-Omics Research Center for Brain Disorders, The First Affiliated Hospital, University of South China, Hengyang, 421001, China.
Transl Neurodegener. 2024 Sep 12;13(1):48. doi: 10.1186/s40035-024-00437-6.
Parkinson's disease (PD) is the second most common neurodegenerative disease. The development of PD is closely linked to genetic and environmental factors, with GBA1 variants being the most common genetic risk. Mutations in the GBA1 gene lead to reduced activity of the coded enzyme, glucocerebrosidase, which mediates the development of PD by affecting lipid metabolism (especially sphingolipids), lysosomal autophagy, endoplasmic reticulum, as well as mitochondrial and other cellular functions. Clinically, PD with GBA1 mutations (GBA1-PD) is characterized by particular features regarding the progression of symptom severity. On the therapeutic side, the discovery of the relationship between GBA1 variants and PD offers an opportunity for targeted therapeutic interventions. In this review, we explore the genotypic and phenotypic correlations, etiologic mechanisms, biomarkers, and therapeutic approaches of GBA1-PD and summarize the current state of research and its challenges.
帕金森病(PD)是第二常见的神经退行性疾病。PD 的发展与遗传和环境因素密切相关,GBA1 变体是最常见的遗传风险因素。GBA1 基因的突变导致编码酶葡糖脑苷脂酶活性降低,通过影响脂质代谢(特别是鞘脂)、溶酶体自噬、内质网以及线粒体和其他细胞功能,从而介导 PD 的发生。临床上,具有 GBA1 突变的 PD(GBA1-PD)的特征在于症状严重程度进展方面具有特定特征。在治疗方面,发现 GBA1 变体与 PD 之间的关系为靶向治疗干预提供了机会。在这篇综述中,我们探讨了 GBA1-PD 的基因型和表型相关性、病因机制、生物标志物和治疗方法,并总结了当前的研究现状及其挑战。