Huang Yi, Chen Xiang, He Xiaofei, Guo Caifeng, Sun Xicui, Liang Fengyin, Long Simei, Lu Xilin, Feng Luyang, Guo Wenyuan, Zeng Yixuan, Pei Zhong
Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, The First Affiliated Hospital, Sun Yat‑sen University, Guangzhou, Guangdong 510080, P.R. China.
Department of Neurology, Guangzhou Huiai Hospital, Guangzhou, Guangdong 510370, P.R. China.
Mol Med Rep. 2017 Jul;16(1):254-262. doi: 10.3892/mmr.2017.6551. Epub 2017 May 9.
Parkinson's disease (PD) is a common neurodegenerative disorder that affects ~2% of the human population aged >65. α‑synuclein serves a role in the pathogenesis of PD as it is a primary component of Lewy bodies, a pathological feature of PD. Endosomal‑lysosomal dysfunction may be a key factor involved in the pathophysiology of PD, and may cause PD‑associated neurodegeneration via α‑synuclein‑dependent and ‑independent mechanisms. The D620N mutation in the endosomal‑lysosomal gene, vacuolar protein sorting‑associated protein 35 (VPS35), has been linked to PD. To clarify the underlying cellular mechanism of the VPS35 D620N mutation in PD, cell growth and endosomal‑lysosomal functions were investigated in Saccharomyces cerevisiae (sc) yeast cells that exhibited various expression levels of scVPS35, in the presence or absence of non‑toxic expression levels of α‑synuclein. Overexpression of the scVPS35 D686N mutation (the yeast equivalent of D620N) did not lead to toxicity in yeast. However, the co‑expression of high copy numbers of scVPS35 D686N and low copy numbers of α‑synuclein caused toxicity, whereas the co‑expression of scVPS35 wild‑type and α‑synuclein did not. In addition, the scVPS35 D686N mutant enhanced α‑synuclein aggregation. Fragmentation of vacuoles and subsequent inhibition of lysosome function was evident in yeast cells bearing the scVPS35 mutant. The results of the present study suggested that α‑synuclein and scVPS35 were interlinked via the endosomal‑lysosome pathway, which is important for the pathogenesis of PD.
帕金森病(PD)是一种常见的神经退行性疾病,影响约2%年龄大于65岁的人群。α-突触核蛋白在PD的发病机制中起作用,因为它是路易小体的主要成分,而路易小体是PD的一种病理特征。内体-溶酶体功能障碍可能是PD病理生理学中的一个关键因素,并可能通过α-突触核蛋白依赖性和非依赖性机制导致与PD相关的神经变性。内体-溶酶体基因空泡蛋白分选相关蛋白35(VPS35)中的D620N突变与PD有关。为了阐明VPS35 D620N突变在PD中的潜在细胞机制,在存在或不存在无毒表达水平的α-突触核蛋白的情况下,对表现出不同表达水平的酿酒酵母(sc)细胞中的细胞生长和内体-溶酶体功能进行了研究。scVPS35 D686N突变体(D620N的酵母等效物)的过表达不会导致酵母中毒。然而,高拷贝数的scVPS35 D686N和低拷贝数的α-突触核蛋白的共表达会导致毒性,而scVPS35野生型和α-突触核蛋白的共表达则不会。此外,scVPS35 D686N突变体增强了α-突触核蛋白的聚集。在携带scVPS35突变体的酵母细胞中,液泡碎片化以及随后溶酶体功能的抑制是明显的。本研究结果表明,α-突触核蛋白和scVPS35通过内体-溶酶体途径相互联系,这对PD的发病机制很重要。