An immunohistochemical study on nuclear bodies in neurodegenerativediseases with abnormal protein aggregates

研究了PML核体和Cajal核体在多系统萎缩（MSA）和散发性肌萎缩性侧索硬化症（ALS）中的运动动力学。在MSA中，在桥核神经元的核内，发现PML小体在r-synuclein阳性的核内包涵体存在下发生变形：圆形结构变为棒状或线状异常结构。统计学上，MSA组（n=5） pml小体总体积明显小于对照组（n=5）。ALS组腰前角细胞核Cajal小体数量（n=5:17.19+/-4.09）明显少于对照组（n=5:17.19+/-4.09）。我们很容易推测，在MSA和ALS中，这些与神经元活力相关的PML和cajal小体的减少分别与r-synuclein和TDP-43的异常有关，在神经元细胞死亡中起重要作用。

The kinetics of two nuclear bodies, PML and Cajal bodies, werestudied in multiple system atrophy (MSA) and sporadic amyotrophic lateral sclerosis (ALS),respectively. In MSA, in the nuclei of the pontine nuclei neurons, PML bodies were found tobe deformed in the presence of r-synuclein-positive intranuclear inclusions: the roundstructures changed to rod-like or linear abnormal ones. Statistically, the entire volume ofPML bodies was significantly decreased in MSA group (n=5) than in control group (n=5). InALS, in the nuclei of the lumbar anterior horn cells, the number of Cajal bodies was significantly decreased in ALS group (n=5: 8.06+/-4.41) than in control group (n=5:17.19+/-4.09). It is tempting to speculate that in MSA and ALS, these decreases of PML andCajal bodies, which are associated with neuronal viability, play an important role inneuronal cell death in relation to abnormalities of r-synuclein and TDP-43, respectively.