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

对多系统萎缩(MSA)和散发性肌萎缩侧索硬化症(ALS)两种核小体PML和Cajal小体的动力学进行了研究。在MSA中，在脑桥核神经元的核中，可见PML小体在r-突触核蛋白阳性的核内包涵体的存在下发生变形：圆形结构变为杆状或线状异常结构。经统计学处理，MSA组(n=5)PML小体体积明显小于对照组(n=5)。在腰椎前角细胞核内，ALS组Cajal小体数目(n=5：8.06+/-4.41)明显少于对照组(n=5：17.19+/-4.09)。推测在MSA和ALS中，与神经元活性有关的PML和Cajal小体的这些减少分别与r-突触核蛋白和TDP-43的异常有关，在神经细胞死亡中起着重要作用。

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.