Mortiboys Heather Jane, Schaefer Jochen, Reichmann Heinz, Jackson Sandra
Department of Neurology, Technical University, Dresden, Germany.
Neurol Neurochir Pol. 2007 Mar-Apr;41(2):150-9.
In recent years much of the research conducted in the field of Parkinson's disease (PD) has been aimed at trying to elucidate the molecular mechanisms involved in the development of the rare familial forms of the disease, with the hope that an understanding of these mechanisms will shed some light into the pathogenesis of the more prevalent idiopathic form of the disease. These studies have implicated mitochondrial dysfunction in the pathogenesis of familial PD, either as a primary event, with disease causing mutations identified in genes encoding mitochondrial proteins, or as a secondary event. Thus, the role played by mitochondrial respiratory chain function in the pathogeneis of idiopathic PD, an area of research that was a main focus some 15-20 years ago, has come back to the forefront of the field. A number of studies have directly investigated respiratory chain function in a variety of tissues from idiopathic PD patients. Although abundant in number, these studies have provided contradictory results, which still require clarification. In this review we will examine the data indicating the presence of a respiratory chain defect in patients with idiopathic PD and attempt to link it with the pathways that have been implicated by familial studies.
近年来，帕金森病（PD）领域的许多研究旨在阐明该疾病罕见家族形式发展过程中涉及的分子机制，希望对这些机制的理解能为更常见的特发性帕金森病的发病机制提供一些线索。这些研究表明线粒体功能障碍在家族性帕金森病的发病机制中起作用，要么作为主要事件，在编码线粒体蛋白的基因中发现致病突变，要么作为次要事件。因此，线粒体呼吸链功能在特发性帕金森病发病机制中的作用，这一约15 - 20年前曾是主要研究焦点的领域，如今又重新回到了该领域的前沿。许多研究直接调查了特发性帕金森病患者各种组织中的呼吸链功能。尽管这类研究数量众多，但结果相互矛盾，仍需进一步阐明。在这篇综述中，我们将审视表明特发性帕金森病患者存在呼吸链缺陷的数据，并尝试将其与家族性研究中涉及的途径联系起来。
