Seychell Raina Marie, El Saghir Adam, Farrugia Gianluca, Vassallo Neville
Department of Physiology and Biochemistry, Faculty of Medicine and Surgery, University of Malta, MSD 2080 Msida, Malta.
Centre for Molecular Medicine and Biobanking, University of Malta, MSD 2080 Msida, Malta.
Membranes (Basel). 2025 May 13;15(5):148. doi: 10.3390/membranes15050148.
Mitochondria possess a double-membrane envelope which is susceptible to insult by pathogenic intracellular aggregates of amyloid-forming peptides, such as the amyloid-beta (1-42) (Aβ42) peptide and the human islet amyloid polypeptide (hIAPP). The molecular composition of membranes plays a pivotal role in regulating peptide aggregation and cytotoxicity. Therefore, we hypothesized that modifying the physicochemical properties of mitochondrial model membranes with a small molecule might act as a countermeasure against the formation of, and damage by, membrane-active amyloid peptides. To investigate this, we inserted the natural ubiquinone Coenzyme Q10 (CoQ10) in model mito-mimetic lipid vesicles, and studied how they interacted with Aβ42 and hIAPP peptide monomers and oligomers. Our results demonstrate that the membrane incorporation of CoQ10 significantly attenuated fibrillization of the peptides, whilst also making the membranes more resilient against peptide-induced permeabilization. Furthermore, these protective effects were linked with the ability of CoQ10 to enhance membrane packing in the inner acyl chain region, which increased the mechanical stability of the vesicle membranes. Based on our collective observations, we propose that mitochondrial resilience against toxic biomolecules implicit in protein misfolding disorders such as Alzheimer's disease and type-2 diabetes, could potentially be enhanced by increasing CoQ10 levels within mitochondria.
线粒体具有双层膜包膜,易受淀粉样蛋白形成肽的致病性细胞内聚集体的损害,例如β淀粉样蛋白(1-42)(Aβ42)肽和人胰岛淀粉样多肽(hIAPP)。膜的分子组成在调节肽聚集和细胞毒性方面起着关键作用。因此,我们推测用小分子修饰线粒体模型膜的物理化学性质可能是对抗膜活性淀粉样肽形成及其造成损害的一种对策。为了对此进行研究,我们将天然泛醌辅酶Q10(CoQ10)插入模拟线粒体的脂质囊泡模型中,并研究它们如何与Aβ42和hIAPP肽单体及寡聚体相互作用。我们的结果表明,CoQ10掺入膜中可显著减弱肽的纤维化,同时还使膜对肽诱导的通透性更具弹性。此外,这些保护作用与CoQ10增强内酰基链区域膜堆积的能力有关,这增加了囊泡膜的机械稳定性。基于我们的总体观察结果,我们提出,通过增加线粒体内CoQ10的水平,可能会增强线粒体对蛋白质错误折叠疾病(如阿尔茨海默病和2型糖尿病)中隐含的有毒生物分子的抵抗力。