Influences of intranuclear inclusion on nuclear size – morphometric study on pontine neurons of neuronal intranuclear inclusion disease cases
Influences of intranuclear inclusion on nuclear size – morphometric study on pontine neurons of neuronal intranuclear inclusion disease cases
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核内包涵体对核大小的影响——神经元核内包涵体病病例脑桥神经元的形态测量研究
DOI：
10.1007/s00401-002-0614-9
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发表时间：
2003
影响因子：
12.7
通讯作者：
T. Hattori
中科院分区：
文献类型：
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作者：
T. Uchihara;J. Tanaka;N. Funata;K. Arai;T. Hattori
抽象的。为了寻找核包涵体(NIS)对人脑神经退行性变的可能影响，我们对3例无关的神经元核内包涵病(NIID)病例和5例对照病例的脑桥神经元的形态特征进行了量化。在脑桥切片上测量各神经元核的横截面积及其畸形指数(长轴/短轴和偏离完美圆的圆形指数)，并对其与NIS的关系进行统计学分析。含泛素免疫阳性神经元核团的横截面积(110.6±1.6)µm2，明显小于对照组(90.5±0.7)µm2。即使从含有NI的核的横截面积(97.4±1.5)微米~2中减去NIS所占的横截面积，这一差异仍然显著。如果在NI存在的情况下加速了核收缩，这很难解释。相反，NIID脑桥神经元中NI的形成可能直接或间接地与一种机制有关，该机制可以抵消而不是加速核收缩。由于即使在NIS患者的神经元中也存在明显的核畸形，其核明显大于对照组，因此核畸形不是继发于其萎缩，而是与核萎缩无关的神经变性的另一个方面。NIS与NIID脑中较大核大小的神经元的联系表明，NIS对神经元不一定是有毒的。
Abstract. In looking for a possible influence of nuclear inclusions (NIs) on neurodegeneration in human brains, we quantified morphological features of pontine neurons of three unrelated cases of neuronal intranuclear inclusion disease (NIID) and five control cases. Cross-sectional area of each neuronal nucleus and the indices for its deformity (long axis/short axis and circularity index defined as deviation from the perfect circle) were measured on pontine sections and their relation to NIs was statistically analyzed. Cross-sectional area of neuronal nuclei harboring ubiquitin-immunopositive NIs was significantly larger (110.6±1.6 µm2, mean ± SE), while that of nuclei not harboring NIs was smaller (77.8±1.5 µm2) than that in controls (90.5±0.7 µm2). This difference remained significant even when the cross-sectional area occupied by NIs was subtracted from that of the nucleus harboring the NI (97.4±1.5 µm2). This could hardly be explained if nuclear shrinkage is accelerated in the presence of NI. On the contrary, NI formation in pontine neurons of NIID might be linked, either directly or indirectly, to a mechanism, which counteracts rather than accelerates nuclear shrinkage. Because nuclear deformity was apparent even in neurons with NIs, whose nuclei were significantly larger than controls, the nuclear deformity is not secondary to its shrinkage and represents another aspect of neurodegeneration independent of nuclear shrinkage. Association of NIs to neurons of larger nuclear size in NIID brain indicates that NIs are not necessarily toxic to neurons.
小鼠 Sca1 基因座中的长 CAG 重复复制了 SCA1 特征，并揭示了突变蛋白溶解度对选择性神经元脆弱性的影响。
DOI：
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发表时间：
2002
期刊：
Neuron 34
影响因子：
--
作者：
Watase;K.
通讯作者：
K.