Immunohistochemical Features of Dystrophic Axons in Papillon Dogs with Neuroaxonal Dystrophy
Immunohistochemical Features of Dystrophic Axons in Papillon Dogs with Neuroaxonal Dystrophy
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DOI：
10.1354/vp.08-vp-0156-u-fl
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发表时间：
2009-05-01
影响因子：
2.4
通讯作者：
Uchida, K.
中科院分区：
文献类型：
--
作者：
Nibe, K.;Nakayama, H.;Uchida, K.
用免疫组织化学方法观察了神经轴索营养不良(NAD)的Papillon犬脑组织中营养不良轴突的免疫组织化学特征，并与1只有小脑皮质缺陷症(CCA)的犬和1只无神经体征的犬进行了比较。组织学上，所有NAD犬的中枢神经系统均可观察到许多营养不良的轴突。这些轴突改变在CCA组和对照组犬中均未见。CCA犬可见严重的浦肯野细胞丢失，而所有NAD犬的皮损较轻。免疫组织化学染色显示，许多营养不良轴突呈神经细丝、tau、α/β-突触核蛋白、HSP70、泛素、突触素、突触素-1和突触体相关蛋白-25(SNAP-25)阳性。少数营养不良轴突呈α-突触核蛋白阳性。此外，这些营养不良的轴突，特别是在薄束核、楔状核、橄榄核和三叉神经脊束中，Calretinin、Calbindin和ParvalBrain这三种钙结合蛋白呈强阳性免疫反应。营养不良轴突中突触相关蛋白的聚集可能表明突触前部分的突触功能障碍。营养不良轴突中α-突触核蛋白的积聚和钙结合蛋白阳性球体的区域特异性出现被认为是NAD的独特特征，为阐明其发病机制提供了关键。
The immunohistochemical features of dystrophic axons in brain tissues of Papillon dogs with neuroaxonal dystrophy (NAD) were examined in comparison with 1 dog with cerebellar cortical abiotrophy (CCA) and a dog without neurologic signs. Histologically, many dystrophic axons were observed throughout the central nervous system of all dogs with NAD. These axonal changes were absent in the dog with CCA and in the control dog. Severe Purkinje cell loss was found in the dog with CCA, whereas the lesions were milder in all dogs with NAD. Immunohistochemically, the many dystrophic axons were positive for neurofilaments, tau, alpha/beta-synuclein, HSP70, ubiquitin, synaptophysin, syntaxin-1, and synaptosomal-associated protein-25 (SNAP-25). A few dystrophic axons were positive for alpha-synuclein. In addition, these dystrophic axons, especially in the nucleus gracilis, cuneatus, olivaris, and spinal tract of the trigeminal nerve, were intensely immunopositive for the 3 calcium-binding proteins calretinin, calbindin, and parvalbumin. The accumulation of synapse-associated proteins in the dystrophic axons may indicate dysfunction of the synapse at the presynaptic portion. The accumulation of alpha-synuclein in the dystrophic axon and region-specific appearance of calcium-binding protein-positive spheroids are considered as unique features in NAD of Papillon dogs, providing the key to elucidate the pathogenesis.