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首页> 外文期刊>Cell and Tissue Research >Ablation of TNAP function compromises myelination and synaptogenesis in the mouse brain.
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Ablation of TNAP function compromises myelination and synaptogenesis in the mouse brain.

机译:消融TNAP功能会影响小鼠脑中的髓鞘系和突触突变。

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Mutations in the tissue-nonspecific alkaline phosphatase (TNAP) gene can result in skeletal and dental hypomineralization and severe neurological symptoms. TNAP is expressed in the synaptic cleft and the node of Ranvier in normal adults. Using TNAP knockout (KO) mice (Akp2(-/-)), we studied synaptogenesis and myelination with light- and electron microscopy during the early postnatal days. Ablation of TNAP function resulted in a significant decrease of the white matter of the spinal cord accompanied by ultrastructural evidence of cellular degradation around the paranodal regions and a decreased ratio and diameter of the myelinated axons. In the cerebral cortex, myelinated axons, while present in wild-type, were absent in the Akp2( -/- ) mice and these animals also displayed a significantly increased proportion of immature cortical synapses. The results suggest that TNAP deficiency could contribute to neurological symptoms related to myelin abnormalities and synaptic dysfunction, among which epilepsy, consistently present in the Akp2(-/-) mice and observed in severe cases of hypophosphatasia.
机译:组织非特异性碱性磷酸酶(TNAP)基因中的突变可导致骨骼和牙齿支质和严重的神经症状。 TNAP在突触裂缝中表示,在普通成年人中的Ranvier的节点中。使用TNAP敲除(KO)小鼠(AKP2( - / - )),我们在出生后早期使用光线和电子显微镜研究了Synaptogens和Myelinal。烧蚀TNAP函数导致脊髓的白质显着降低,伴随着剖腹区域周围细胞降解的超微结构证据和脊髓轴突的减少和直径。在脑皮质中,在Akp2( - / - )小鼠中不存在于野生型中的髓鞘轴颈,并且这些动物也显示出显着增加的未成熟皮质突触比例。结果表明,TNAP缺乏可能导致与髓鞘异常和突触功能障碍相关的神经系统症状,其中癫痫症中的癫痫症,始终存在于AKP2( - / - )小鼠中,并且在严重的次磷酸盐症中观察到。

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