alpha-Synucleinopathy phenotypes.
alpha-Synucleinopathy phenotypes.
复制标题
DOI：
10.1016/s1353-8020(13)70017-8
复制
发表时间：
2014-01-01
影响因子：
4.1
通讯作者：
Halliday, Glenda M
中科院分区：
文献类型：
--
作者：
McCann, Heather;Stevens, Claire H;Halliday, Glenda M
α-突触核蛋白病是以神经元、神经纤维或神经胶质细胞中α-突触核蛋白聚集体的异常积累为特征的神经变性疾病。虽然少量的这些α-突触核蛋白病理可能发生在一些没有相关神经变性的神经学正常个体中，但这些个体中没有神经变性排除了他们患有退行性α-突触核蛋白病，并且尚未确定这些个体是否具有临床前疾病的形式。有三种主要类型的α-突触核蛋白病，帕金森病（PD）、路易体痴呆（DLB）和多系统萎缩（MSA），其他罕见疾病也具有α-突触核蛋白病理学，如各种神经轴突营养不良。三种主要的α-突触核蛋白病中的每一种都存在多种临床表型，这些表型在其基础神经病理学的动态分布中不同。确定导致不同α-突触核蛋白表型的因素可能最终导致更有针对性的治疗以及更准确的临床预后。
alpha-Synucleinopathies are neurodegenerative diseases characterised by the abnormal accumulation of alpha-synuclein aggregates in neurons, nerve fibres or glial cells. While small amounts of these alpha-synuclein pathologies can occur in some neurologically normal individuals who do not have associated neurodegeneration, the absence of neurodegeneration in such individuals precludes them from having a degenerative alpha-synucleinopathy, and it has yet to be established whether such individuals have a form of preclinical disease. There are three main types of alpha-synucleinopathy, Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), with other rare disorders also having alpha-synuclein pathologies, such as various neuroaxonal dystrophies. Multiple clinical phenotypes exist for each of the three main alpha-synucleinopathies, with these phenotypes differing in the dynamic distribution of their underlying neuropathologies. Identifying the factors involved in causing different alpha-synuclein phenotypes may ultimately lead to more targeted therapeutics as well as more accurate clinical prognosis.