Mitochondrial Dysfunction and Susceptibility to Parkinson's disease: New Models of Pathogenetic Interactions

帕金森氏病是仅次于阿尔茨海默氏病的第二种最常见的神经退行性疾病。在英国，它影响了大约150，000 - 200，000人，这在降低患者和护理人员的生活质量方面是一个重大负担。帕金森病的病因尚未完全了解，但对家族性帕金森病的病因有许多见解，这些见解为可能导致影响大多数人的疾病的机制提供了有价值的见解。帕金森病的遗传原因包括PINK 1基因的异常。已经发现PINK 1参与细胞内的能量产生，也对其他重要过程如自由基的产生有反应。这项资助涉及来自英国、德国、意大利和加拿大的世界四个实验室。所有这些都主要关注PINK 1功能。他们聚集在一起，在他们的研究中合作，以确定PINK 1缺乏如何使个体容易患上帕金森病，以及这种缺乏如何与其他导致帕金森病的遗传异常相互作用。重点在于鉴定分子事件的序列，PINK 1缺陷的结果，以鉴定可能适合于药物干预以减缓或预防PINK 1相关细胞异常进展的潜在靶标。然后将在帕金森病的其他模型中评估这些靶点，特别是在特发性帕金森病中研究它们的适用性。

Parkinson's disease is the second most common neurodegenerative disorder after Alzheimer's disease. It affects approximately 150,000-200,000 in the UK and that represents a significant burden in terms of reduced quality of life of patient and carer. The cause of Parkinson's disease is not yet completely understood, but there are many insights into causes of familial Parkinson's disease which have provided valuable insight into those mechanisms that may result in the disease affecting the majority of people. The genetic causes of Parkinson's disease include abnormalities of the PINKl gene. PINKl has been found to be involved in energy production within the cell, there is also response for other important processes such as the generation of free radicals. This grant involves four laboratories in the world from the UK, Germany, Italy and Canada. All of which have a major focus of interest on PINKl function. They have come together to collaborate in their research to identify how PINKl deficiency makes individuals susceptible to the development of Parkinson's disease and how this deficiency might also interact with other genetic abnormalities that cause Parkinson's disease. The focus is on identifying the sequence of molecular events, the results from PINKl deficiency to identify potential targets that may be suitable for drug intervention to slow or prevent progression of the PINKl related cell abnormalities. These targets will then be assessed in other models of Parkinson's disease and particularly for their suitability to be studied in idiopathic Parkinson's disease.