Zorzano Antonio, Claret Marc
Molecular Medicine Program, Institute of Research in Biomedicine (IRB Barcelona) Barcelona, Spain ; Departament de Bioquímica i Biologia Molecular, Facultat de Biologia, Universitat de Barcelona Barcelona, Spain ; CIBER de Diabetes y Enfermedades Metabólicas Asociadas, Instituto de Salud Carlos III Barcelona, Spain.
CIBER de Diabetes y Enfermedades Metabólicas Asociadas, Instituto de Salud Carlos III Barcelona, Spain ; Diabetes and Obesity Research Laboratory, Institut d'Investigacions Biomèdiques August Pi i Sunyer Barcelona, Spain.
Front Aging Neurosci. 2015 Jun 10;7:101. doi: 10.3389/fnagi.2015.00101. eCollection 2015.
Mitochondrial dynamics is a term that encompasses the movement of mitochondria along the cytoskeleton, regulation of their architecture, and connectivity mediated by tethering and fusion/fission. The importance of these events in cell physiology and pathology has been partially unraveled with the identification of the genes responsible for the catalysis of mitochondrial fusion and fission. Mutations in two mitochondrial fusion genes (MFN2 and OPA1) cause neurodegenerative diseases, namely Charcot-Marie Tooth type 2A and autosomal dominant optic atrophy (ADOA). Alterations in mitochondrial dynamics may be involved in the pathophysiology of prevalent neurodegenerative conditions. Moreover, impairment of the activity of mitochondrial fusion proteins dysregulates the function of hypothalamic neurons, leading to alterations in food intake and in energy homeostasis. Here we review selected findings in the field of mitochondrial dynamics and their relevance for neurodegeneration and hypothalamic dysfunction.
线粒体动力学是一个术语,涵盖了线粒体沿细胞骨架的移动、其结构的调节以及由拴系和融合/裂变介导的连接性。随着负责线粒体融合和裂变催化的基因的鉴定,这些事件在细胞生理学和病理学中的重要性已部分得以揭示。两个线粒体融合基因(MFN2和OPA1)的突变会导致神经退行性疾病,即2A型夏科-马里-图斯病和常染色体显性遗传性视神经萎缩(ADOA)。线粒体动力学的改变可能参与常见神经退行性疾病的病理生理学过程。此外,线粒体融合蛋白活性的受损会使下丘脑神经元的功能失调,导致食物摄入和能量稳态的改变。在此,我们综述线粒体动力学领域的部分研究发现及其与神经退行性变和下丘脑功能障碍的相关性。