Postnatal brain and skull growth in an Apert syndrome mouse model.
Postnatal brain and skull growth in an Apert syndrome mouse model.
复制标题
DOI：
10.1002/ajmg.a.35805
复制
发表时间：
2013-04
影响因子：
2
通讯作者：
Aldridge, Kristina
中科院分区：
文献类型：
--
作者：
Hill, Cheryl A.;Martinez-Abadias, Neus;Motch, Susan M.;Austin, Jordan R.;Wang, Yingli;Jabs, Ethylin Wang;Richtsmeier, Joan T.;Aldridge, Kristina
关键词：
颅面和神经组织在产前和产后生长过程中协调发育。 FGFR 相关的颅缝早闭综合征，例如阿佩尔综合征 (AS)，与涉及颅骨和大脑的特定表型相关。我们使用 Fgfr2+/P253R 小鼠分析了 FGFR P253R 突变对阿佩尔综合征的影响，以评估该突变在从出生当天 (P0) 到出生后第 2 天 (P2) 的发育过程中对这两种组织的影响。在 P0 (N=28) 和 P2 (N=23) 时从 Fgfr2+/P253R 小鼠和未受影响的同窝小鼠获取三维磁共振显微镜和计算机断层扫描图像。统计比较组间 23 个头骨和 15 个脑标志的 3D 坐标数据。结果表明，与未受影响的同窝小鼠相比，Fgfr2+/P253R 小鼠的面部骨骼和大脑生长减少，而颅骨和尾部区域的高度和宽度生长增加。头骨和大脑中差异生长模式的这种局部对应表明它们在发育过程中持续相互作用，并表明这两种组织相对于未受影响的同窝动物表现出不同的出生后生长模式。然而，颅脑关系从 P0 到 P2 的变化意味着受突变影响的每个组织都保留了一定程度的独立性，而不是一个组织指导另一个组织的发育。
Craniofacial and neural tissues develop in concert throughout pre- and postnatal growth. FGFR-related craniosynostosis syndromes, such as Apert syndrome (AS), are associated with specific phenotypes involving both the skull and the brain. We analyzed the effects of the FGFR P253R mutation for Apert syndrome using the Fgfr2+/P253R mouse to evaluate the effects of this mutation on these two tissues over the course of development from day of birth (P0) to postnatal day 2 (P2). Three-dimensional magnetic resonance microscopy and computed tomography images were acquired from Fgfr2+/P253R mice and unaffected littermates at P0 (N=28) and P2 (N=23). 3D coordinate data for 23 skull and 15 brain landmarks were statistically compared between groups. Results demonstrate that the Fgfr2+/P253R mice show reduced growth in the facial skeleton and the cerebrum, while the height and width of the neurocranium and caudal regions of the brain show increased growth relative to unaffected littermates. This localized correspondence of differential growth patterns in skull and brain point to their continued interaction through development and suggest that both tissues display divergent postnatal growth patterns relative to unaffected littermates. However, the change in the skull-brain relationship from P0 to P2 implies that each tissue affected by the mutation retains a degree of independence, rather than one tissue directing the development of the other.
登录
查看更多内容
影响因子：
2.9
作者：
Ever, Leah;Zhao, Rui-jing;Gaiano, Nicholas
通讯作者：
Gaiano, Nicholas
影响因子：
3.3
作者：
CHEVERUD, JM
通讯作者：
CHEVERUD, JM
影响因子：
2.9
作者：
CHEVERUD, JM
通讯作者：
CHEVERUD, JM
影响因子：
4.1
作者：
Chen, L;Li, D;Deng, CX
通讯作者：
Deng, CX
影响因子：
--
作者：
DELEON, GA;DELEON, G;ALBURGER, PD
通讯作者：
ALBURGER, PD