Reduced Purkinje cell dendritic arborization and loss of dendritic spines in essential tremor

Reduced Purkinje cell dendritic arborization and loss of dendritic spines in essential tremor
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DOI:
10.1093/brain/awu314
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发表时间:
2014-12-01
期刊:
影响因子:
14.5
通讯作者:
Faust, Phyllis L.
Faust, Phyllis L.
中科院分区:
医学1区
文献类型:
--
作者:
Louis, Elan D.;Lee, Michelle;Faust, Phyllis L.

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基于积累的特发性震颤浦肯野细胞生物学异常的死后证据,我们假设,与年龄匹配的对照组相比,特发性震颤病例中浦肯野细胞群中的树突形态退行性变化将是明显的。用Golgi-Kopsch方法对27例原发性震颤患者和27例年龄匹配的对照者的小脑皮质组织进行了处理。浦肯野细胞树突状解剖定量使用Neurolucida显微镜系统与电动阶段。在所有的措施,特发性震颤的情况下,表现出显着减少树突的复杂性相比,控制。特发性震颤病例与对照组的中位值为:5712.1对10 403.2亩(总树突长度,P = 0.01),465.9与592.5 μ m(分支长度,P = 0.01),22.5与29.0(最大分支顺序,P = 0.001),165.3与311.7(终止数量,P = 0.008)。此外,树突棘密度在特发性震颤病例中降低(中位数= 0.82对1.02 μ m(-1),P = 0.03)。我们的演示的回归性变化,在原发性震颤相对于控制大脑的浦肯野细胞树突状结构和棘提供了额外的证据浦肯野细胞生物学的普遍异常,在这种疾病中,影响多个神经元细胞室,包括他们的轴突,细胞体,树突和棘。
Based on accumulating post-mortem evidence of abnormalities in Purkinje cell biology in essential tremor, we hypothesized that regressive changes in dendritic morphology would be apparent in the Purkinje cell population in essential tremor cases versus age-matched controls. Cerebellar cortical tissue from 27 cases with essential tremor and 27 age-matched control subjects was processed by the Golgi-Kopsch method. Purkinje cell dendritic anatomy was quantified using a Neurolucida microscopic system interfaced with a motorized stage. In all measures, essential tremor cases demonstrated significant reductions in dendritic complexity compared with controls. Median values in essential tremor cases versus controls were: 5712.1 versus 10 403.2 mu m (total dendrite length, P = 0.01), 465.9 versus 592.5 mu m (branch length, P = 0.01), 22.5 versus 29.0 (maximum branch order, P = 0.001), and 165.3 versus 311.7 (number of terminations, P = 0.008). Furthermore, the dendritic spine density was reduced in essential tremor cases (medians = 0.82 versus 1.02 mu m(-1), P = 0.03). Our demonstration of regressive changes in Purkinje cell dendritic architecture and spines in essential tremor relative to control brains provides additional evidence of a pervasive abnormality of Purkinje cell biology in this disease, which affects multiple neuronal cellular compartments including their axon, cell body, dendrites and spines.