Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
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DOI：
10.1073/pnas.94.10.5401
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发表时间：
1997-05-13
影响因子：
11.1
通讯作者：
Greenough, WT
中科院分区：
文献类型：
--
作者：
Comery, TA;Harris, JB;Greenough, WT
脆性 X 综合征是由脆性 X 智力迟钝蛋白 (FMRP) 的表达受阻引起的，脆性 X 患者的高尔基体成熟的大脑皮层表现出长、细、曲折的突触后棘，类似于正常早期新皮质发育过程中观察到的棘。在这里，我们描述了缺乏该蛋白表达的转基因脆性X基因（Fmr1）敲除小鼠的高尔基体浸渍大脑皮层中的树突棘，脆性X基因敲除小鼠枕叶皮层V层锥体细胞顶端树突上的树突棘比野生型小鼠的更长，并且通常薄而曲折，与人类综合症相似，表明FMRP表达是正常脊柱形态发育所必需的。此外，基因敲除小鼠沿顶端树突的棘密度更大，这可能反映了突触稳定和消除或修剪的发育组织过程受损。
Fragile X syndrome arises from blocked expression of the fragile X mental retardation protein (FMRP), Golgi-impregnated mature cerebral cortex from fragile X patients exhibits long, thin, tortuous postsynaptic spines resembling spines observed during normal early neocortical development. Here we describe dendritic spines in Golgi-impregnated cerebral cortex of transgenic fragile X gene (Fmr1) knockout mice that lack expression of the protein, Dendritic spines on apical dendrites of layer V pyramidal cells in occipital cortex of fragile X knockout mice were longer than those in wild-type mice and were often thin and tortuous, paralleling the human syndrome and suggesting that FMRP expression is required for normal spine morphological development. Moreover, spine density along the apical dendrite was greater in the knockout mice, which may reflect impaired developmental organizational processes of synapse stabilization and elimination or pruning.