Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies.
Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies.
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软脑膜基底膜的破裂与先天性肌营养不良小鼠模型中齿状回发育缺陷有关
DOI：
10.1016/j.neulet.2011.09.040
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发表时间：
2011-11-07
影响因子：
2.5
通讯作者：
Li X
中科院分区：
文献类型：
--
作者：
Li J;Yu M;Feng G;Hu H;Li X
先天性肌营养不良症（CMD）的一个子集具有中枢神经系统表现。对于这些CMD有良好的小鼠模型，包括POMGnT1敲除、POMT2敲除和Largemyd小鼠，它们都表现出齿状回缺陷。目前尚不清楚异常的齿状回是如何在发育过程中形成的。在这项研究中，我们进行了详细的形态学检查的成年和新生儿POMGnT1基因敲除，POMT2基因敲除，和Largemyd小鼠的齿状回免疫荧光染色和电子显微镜分析。我们观察到覆盖在齿状回上的软膜基底膜被破坏，颗粒细胞前体细胞通过破坏的软膜基底膜异位。除此之外，敲除齿状回在这些小鼠模型中表现出反应性胶质增生。因此，在先天性肌营养不良症小鼠模型中，软脑膜基底膜的破裂与齿状回发育缺陷有关。
A subset of congenital muscular dystrophies (CMDs) has central nervous system manifestations. There are good mouse models for these CMDs that include POMGnT1 knockout, POMT2 knockout and Largemyd mice with all exhibit defects in dentate gyrus. It is not known how the abnormal dentate gyrus is formed during development. In this study, we conducted a detailed morphological examination of the dentate gyrus in adult and newborn POMGnT1 knockout, POMT2 knockout, and Largemyd mice by immunofluorescence staining and electron microscopic analyses. We observed that the pial basement membrane overlying the dentate gyrus was disrupted and there was ectopia of granule cell precursors through the breached pial basement membrane. Besides these, the knockout dentate gyrus exhibited reactive gliosis in these mouse models. Thus, breaches in the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies.
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DOI：
10.1083/jcb.122.4.809
发表时间：
1993-08
期刊：
The Journal of cell biology
影响因子：
--
作者：
Ervasti JM;Campbell KP
通讯作者：
Campbell KP
影响因子：
2.6
作者：
Liu, JM;Zhang, L;Hu, HY
通讯作者：
Hu, HY
影响因子：
3.5
作者：
Brockington, M;Yuva, Y;Muntoni, F
通讯作者：
Muntoni, F
影响因子：
64.8
作者：
Michele, DE;Barresi, R;Campbell, KP
通讯作者：
Campbell, KP
影响因子：
9.8
作者：
Brockington, M;Blake, DJ;Muntoni, F
通讯作者：
Muntoni, F