Laboratory of Neurophysiology and Plasticity, IRCCS Fondazione Santa Lucia, 00143 Rome, Italy.
Saint Camillus International University of Health and Medical Sciences, 00131 Rome, Italy.
Int J Mol Sci. 2023 Apr 13;24(8):7221. doi: 10.3390/ijms24087221.
Strong evidence suggests a correlation between degeneration and mitochondrial deficiency. Typical cases of degeneration can be observed in physiological phenomena (i.e., ageing) as well as in neurological neurodegenerative diseases and cancer. All these pathologies have the dyshomeostasis of mitochondrial bioenergy as a common denominator. Neurodegenerative diseases show bioenergetic imbalances in their pathogenesis or progression. Huntington's chorea and Parkinson's disease are both neurodegenerative diseases, but while Huntington's disease is genetic and progressive with early manifestation and severe penetrance, Parkinson's disease is a pathology with multifactorial aspects. Indeed, there are different types of Parkinson/Parkinsonism. Many forms are early-onset diseases linked to gene mutations, while others could be idiopathic, appear in young adults, or be post-injury senescence conditions. Although Huntington's is defined as a hyperkinetic disorder, Parkinson's is a hypokinetic disorder. However, they both share a lot of similarities, such as neuronal excitability, the loss of striatal function, psychiatric comorbidity, etc. In this review, we will describe the start and development of both diseases in relation to mitochondrial dysfunction. These dysfunctions act on energy metabolism and reduce the vitality of neurons in many different brain areas.
有强有力的证据表明退化与线粒体功能缺陷之间存在相关性。在生理现象(如衰老)以及神经退行性疾病和癌症中,都可以观察到典型的退化病例。所有这些病理都以线粒体生物能量的动态平衡失调为共同特征。神经退行性疾病在其发病机制或进展中表现出生物能量失衡。亨廷顿舞蹈病和帕金森病都是神经退行性疾病,但亨廷顿舞蹈病是一种遗传性的、进行性的疾病,早期表现明显且具有严重的外显性,而帕金森病则是一种具有多种因素的病理学。事实上,帕金森病有不同的类型。许多形式是与基因突变相关的早发型疾病,而其他的可能是特发性的,在年轻人中出现,或因受伤后衰老导致。虽然亨廷顿舞蹈病被定义为一种多动障碍,但帕金森病是一种运动障碍。然而,它们有很多相似之处,如神经元兴奋性、纹状体功能丧失、精神共病等。在这篇综述中,我们将描述这两种疾病与线粒体功能障碍相关的起始和发展。这些功能障碍作用于能量代谢,降低了许多不同脑区神经元的活力。