Embryonic craniofacial bone volume and bone mineral density in Fgfr2(+/P253R) and nonmutant mice.

Embryonic craniofacial bone volume and bone mineral density in Fgfr2(+/P253R) and nonmutant mice.
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DOI:
10.1002/dvdy.24095
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发表时间:
2014-04
影响因子:
2.5
通讯作者:
Richtsmeier, Joan T.
Richtsmeier, Joan T.
中科院分区:
生物学3区
文献类型:
--
作者:
Percival, Christopher J.;Huang, Yuan;Jabs, Ethylin Wang;Li, Runze;Richtsmeier, Joan T.

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在早期成骨过程中量化个体颅面骨的多个表型方面说明了典型骨生长和成熟的差异,并为理解与疾病相关的突变的局部和整体影响提供了基础。我们量化了早期颅面成骨过程中骨生长和成熟的典型模式,并确定了Fgfr 2 +/P253 R Apert综合征小鼠中这种模式是如何改变的。膜内骨和软骨内骨之间典型的相对骨密度增加的早期差异被注意到,软骨内骨通常在产前时期成熟得更快。几个颅面骨,包括Fgfr 2 +/P253 R小鼠的面骨,显示较低的体积在成骨的最早期和较低的相对密度,直到围产期相对于未受影响的同窝仔。骨体积的估计值和描述形态的线性测量值不一定是协变的,这突出了在探索致病突变的影响时量化大体骨表型的多个方面的价值。成骨机制的差异可能是膜内和软骨内相对密度增加差异的基础。FGFR 2 P253 R突变对骨体积的影响在产前和出生后都会发生变化,而对相对骨密度的影响则更为稳定。
Quantifying multiple phenotypic aspects of individual craniofacial bones across early osteogenesis illustrates differences in typical bone growth and maturation and provides a basis for understanding the localized and overall influence of mutations associated with disease. We quantify the typical pattern of bone growth and maturation during early craniofacial osteogenesis and determine how this pattern is modified in Fgfr2+/P253R Apert syndrome mice. Early differences in typical relative bone density increase are noted between intramembranous and endochondral bones, with endochondral bones normally maturing more quickly during the prenatal period. Several craniofacial bones, including the facial bones of Fgfr2+/P253R mice, display lower volumes during the earliest days of osteogenesis and lower relative densities until the perinatal period relative to unaffected littermates. Estimates of bone volume and linear measures describing morphology do not necessarily covary, highlighting the value of quantifying multiple facets of gross osteological phenotypes when exploring the influence of a disease causing mutation. Differences in mechanisms of osteogenesis likely underlie differences in intramembranous and endochondral relative density increase. The influence of the FGFR2 P253R mutation on bone volume changes across the prenatal period and again after birth, while its influence on relative bone density is more stable.
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