Sarhadi Tanveera Rounaque, Joshi Neha, Nagotu Shirisha
Organelle Biology and Cellular Ageing Lab, Department of Biosciences and Bioengineering, Indian Institute of Technology Guwahati, Guwahati, 781039, Assam, India.
Mol Neurobiol. 2025 Jul 24. doi: 10.1007/s12035-025-05236-2.
Aggregation of α-Synuclein is a hallmark characteristic of Parkinson's disease. Several mutant variants of α-Synuclein linked to the disease that exhibit distinct patterns of localization, aggregation dynamics, and cellular toxicity have been reported. This variability underscores the need for a detailed study of each mutant variant to understand the disease pathology better. While mitochondria have been extensively studied for their role in Parkinson's disease pathogenesis, much less is known about peroxisomes, organelles that share important functions with mitochondria. To address this, we investigated the effect of expression of WT- α-Synuclein and two disease variants A53T and A29S in yeast. Interestingly, the expression of the A53T α-Synuclein variant resulted in a significant increase in peroxisome number per cell. This increase was also observed in cells lacking peroxisome fission proteins Vps1 and Dnm1. Our data also suggests a link between the aggregation propensity of α-Synuclein and the observed effect on peroxisome number. Expression of the A29S variant of α-Synuclein and WT-α-Synuclein which exhibits a lower aggregation propensity than A53T, did not result in a similar increase in peroxisome number. In line with this, enhanced mitochondrial fragmentation was observed only upon expression of A53T α-Synuclein in WT yeast cells.
α-突触核蛋白的聚集是帕金森病的一个标志性特征。已报道了几种与该疾病相关的α-突触核蛋白突变变体,它们表现出不同的定位模式、聚集动力学和细胞毒性。这种变异性凸显了对每个突变变体进行详细研究以更好地理解疾病病理的必要性。虽然线粒体在帕金森病发病机制中的作用已得到广泛研究,但对于与线粒体具有重要共同功能的过氧化物酶体却知之甚少。为了解决这个问题,我们研究了野生型α-突触核蛋白以及两种疾病变体A53T和A29S在酵母中的表达效果。有趣的是,A53Tα-突触核蛋白变体的表达导致每个细胞中的过氧化物酶体数量显著增加。在缺乏过氧化物酶体分裂蛋白Vps1和Dnm1的细胞中也观察到了这种增加。我们的数据还表明α-突触核蛋白的聚集倾向与观察到的对过氧化物酶体数量的影响之间存在联系。α-突触核蛋白的A29S变体和野生型α-突触核蛋白(其聚集倾向低于A53T)的表达并未导致过氧化物酶体数量出现类似增加。与此一致的是,仅在野生型酵母细胞中表达A53Tα-突触核蛋白时才观察到线粒体碎片化增强。