FINE-STRUCTURE OF CEREBELLAR CORTEX IN MENKES KINKY-HAIR DISEASE - X-CHROMOSOME-LINKED COPPER MALABSORPTION
FINE-STRUCTURE OF CEREBELLAR CORTEX IN MENKES KINKY-HAIR DISEASE - X-CHROMOSOME-LINKED COPPER MALABSORPTION
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DOI：
10.1001/archneur.1977.00500130072014
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发表时间：
1977-01-01
影响因子：
--
通讯作者：
GHATAK, NR
中科院分区：
文献类型：
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作者：
HIRANO, A;LLENA, JF;GHATAK, NR
本文对2例x染色体连锁铜吸收不良（Menkes病）患者的小脑皮质进行了光镜和电镜检查，光镜下可见明显的体芽和奇形怪状的树枝状树突。在精细结构水平上，虽然幸存的颗粒细胞已经下降，但浦肯野细胞的特征是存在体细胞棘。大多数棘是突触复合体的一部分，但有些不与任何突触前元件相连。在树枝状树干上也观察到类似的刺。星形胶质细胞增生和非特异性内皮细胞改变。
The cerebellar cortices in 2 patients with X-chromosome-linked Cu malabsorption (Menkes kinky-hair disease) were examined by light microscopy and EM. Somatic sprouts and grotesque dendritic arborization were among the obvious light microscopical changes. At the fine structural level, the Purkinje cells were characterized by the presence of somatic spines although the surviving granule cells had already descended. Most spines were parts of synaptic complexes, but some were unattached to any presynaptic element. Similar spines were also observed on dendritic trunks. Astrocytic gliosis and nonspecific endothelial cell changes were noted.