Mitochondria in neurodegeneration: Investigating the role of mitochondria and metabolism in the cause and therapeutic targeting of neurodegenerative diseases

我们最活跃的组织，如大脑和心脏，在人类生命的几十年里的生存提出了一个非凡的生物学挑战。被称为线粒体的细胞器对这些组织的生死至关重要。在提供这些细胞所需的大量能量和缓冲细胞质钙通量的同时，它们也产生许多导致细胞损伤的分子，并容纳一系列细胞死亡诱导因子。因此，这些细胞器需要大量的维护和质量控制过程。线粒体内稳态的失败与年龄相关的疾病（如神经变性）密切相关。我们的小组旨在了解线粒体稳态与神经退行性疾病（如帕金森病和运动神经元疾病）相关的机制。我们结合了强大的果蝇遗传技术和哺乳动物细胞的分子、细胞生物学和生化方法。对这些机制的深入了解将有助于更好地理解线粒体在神经系统健康和功能障碍中的生理作用，并将有助于指导治疗发展，以对抗神经退行性疾病。

The survival of our most active tissues, such as the brain and heart, throughout decades of a human lifespan presents an extraordinary biological challenge. The cellular organelles known as mitochondria are central to the life and death of these tissues. While providing the high amount of energy required by these cells and buffering cytoplasmic calcium flux, they also produce many of the molecules that cause cellular damage and house an array of cell death-inducing factors. Thus, these organelles require extensive maintenance and quality control processes. Failure in mitochondrial homeostasis is strongly linked to age-related conditions such as neurodegeneration.Our group aims to understand the mechanisms of mitochondrial homeostasis in relation to neurodegenerative diseases such as Parkinson’s disease and motor neuron disease. We use a combination of the powerful genetic techniques of Drosophila (fruit flies) and molecular, cell biology and biochemical approaches in mammalian cells. Insights into these mechanisms will deliver a greater understanding of the role of mitochondrial maintenance in the health and dysfunction of the nervous system in a physiological context and will help guide therapeutic development to combat neurodegenerative diseases.