Inserm UMR 1078, Université de Bretagne Occidentale, Faculté de Médecine et des Sciences de la Santé; Etablissement Français du Sang (EFS) Bretagne; CHRU Brest, Hôpital Morvan, Laboratoire de Génétique Moléculaire, Brest, France.
Yeast Genetics Laboratory, Department of Biology, Maynooth University, Maynooth, County Kildare, Ireland.
Sci Rep. 2016 Sep 16;6:32117. doi: 10.1038/srep32117.
6AP and GA are potent inhibitors of yeast and mammalian prions and also specific inhibitors of PFAR, the protein-folding activity borne by domain V of the large rRNA of the large subunit of the ribosome. We therefore explored the link between PFAR and yeast prion [PSI(+)] using both PFAR-enriched mutants and site-directed methylation. We demonstrate that PFAR is involved in propagation and de novo formation of [PSI(+)]. PFAR and the yeast heat-shock protein Hsp104 partially compensate each other for [PSI(+)] propagation. Our data also provide insight into new functions for the ribosome in basal thermotolerance and heat-shocked protein refolding. PFAR is thus an evolutionarily conserved cell component implicated in the prion life cycle, and we propose that it could be a potential therapeutic target for human protein misfolding diseases.
6AP 和 GA 是酵母和哺乳动物朊病毒的有效抑制剂,也是核糖体大亚基的大 rRNA 结构域 V 所具有的蛋白折叠活性(PFAR)的特异性抑制剂。因此,我们使用 PFAR 富集突变体和定点甲基化探索了 PFAR 和酵母朊病毒 [PSI(+)] 之间的联系。我们证明 PFAR 参与了 [PSI(+)] 的传播和从头形成。PFAR 和酵母热休克蛋白 Hsp104 部分补偿了彼此在 [PSI(+)] 传播中的作用。我们的数据还为核糖体在基础耐热性和热激蛋白重折叠中的新功能提供了深入了解。因此,PFAR 是一种进化上保守的细胞成分,涉及朊病毒生命周期,我们提出它可能是人类蛋白质错误折叠疾病的潜在治疗靶点。