NEUROPATHOLOGY OF GRACILE AXONAL DYSTROPHY (GAD) MOUSE - AN ANIMAL-MODEL OF CENTRAL DISTAL AXONOPATHY IN PRIMARY SENSORY NEURONS
NEUROPATHOLOGY OF GRACILE AXONAL DYSTROPHY (GAD) MOUSE - AN ANIMAL-MODEL OF CENTRAL DISTAL AXONOPATHY IN PRIMARY SENSORY NEURONS
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DOI：
10.1007/bf00294664
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发表时间：
1989-01-01
影响因子：
12.7
通讯作者：
TOMITA, T
中科院分区：
文献类型：
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作者：
MUKOYAMA, M;YAMAZAKI, K;TOMITA, T
一种新的神经系统突变小鼠表现出纤细轴突营养不良（GAD）。退化性病变的发展，出生后80天，首先出现在最喙部的薄束。然后病变向尾侧延伸，累及整个股薄肌束。许多轴索萎缩（营养不良）也出现在退行性病变中，与其严重程度成比例。临床表现与这些病理变化一致，其特征为震颤、共济失调和运动困难。这些开始于第80天左右，并逐渐发展到第150天左右死亡。腰后根及其脊神经节和周围神经正常。电镜下可见营养不良的轴突内充满神经丝、线粒体和管泡状结构。这些可能反映了轴突运输的一些停滞。病变的分布表明，GAD小鼠有一个中央远端轴突病变涉及初级感觉神经元的腰背根神经节。
A new neurological mutant mouse shows a gracile axonal dystrophy (GAD). The degenerative lesion develops by postnatal day 80, first appearing in the most rostral portion of the gracile fascicles. This lesion then extends caudally to involve the entire gracile fascicles. Many axonal swellings (dystrophies) also appear in the degenerative lesions in proportion to their severity. The clinical findings develop in keeping with these pathological changes, and are characterized by tremor, ataxia and difficulty in moving the hid lims. These start around day 80, and progress gradually to death about day 150. The lumbar dorsal roots, their spinal root ganglia and peripheral nerves are normal. Electron microscopic study shows dystrophic axons packed with neurofilaments, mitochondria and tubulovesicular structures. These may reflect some stagnation of axonal transport. The distribution of the lesions suggest that the GAD mouse has a central distal axonopathy involving primary sensory neurons of the lumbar dorsal root ganglia.