Irizarry M C, Growdon W, Gomez-Isla T, Newell K, George J M, Clayton D F, Hyman B T
Alzheimer's Disease Research Unit, Massachusetts General Hospital-East, Charlestown 02129, USA.
J Neuropathol Exp Neurol. 1998 Apr;57(4):334-7. doi: 10.1097/00005072-199804000-00005.
A mutation in the alpha-synuclein gene has recently been linked to some cases of familial Parkinson's disease (PD). We characterized the expression of this presynaptic protein in the midbrain, striatum, and temporal cortex of control, PD, and dementia with Lewy bodies (DLB) brain. Control brain showed punctate pericellular immunostaining. PD brain demonstrated alpha-synuclein immunoreactivity in nigral Lewy bodies, pale bodies and abnormal neurites. Rare neuronal soma in PD brain were immunoreactive for alpha-synuclein. DLB cases demonstrated these findings as well as alpha-synuclein immunoreactivity in cortical Lewy bodies and CA2-3 neurites. These results suggest that, even in sporadic cases, there is an early and direct role for alpha-synuclein in the pathogenesis of PD and the neuropathologically related disorder DLB.
α-突触核蛋白基因的突变最近被认为与某些家族性帕金森病(PD)病例有关。我们对该突触前蛋白在对照、PD和路易体痴呆(DLB)患者脑的中脑、纹状体和颞叶皮质中的表达进行了特征描述。对照脑显示出点状的细胞周免疫染色。PD脑在黑质路易小体、苍白小体和异常神经突中表现出α-突触核蛋白免疫反应性。PD脑中罕见的神经元胞体对α-突触核蛋白有免疫反应性。DLB病例也表现出这些结果,以及皮质路易小体和CA2 - 3神经突中的α-突触核蛋白免疫反应性。这些结果表明,即使在散发性病例中,α-突触核蛋白在PD和神经病理学相关疾病DLB的发病机制中也具有早期直接作用。