小鼠胚胎发育过程中FMRP、FXR1P和FXR2P的免疫细胞化学特征

Yolanda de Diego Otero, Cathy E. Bakker, Prawien Raghoe, Lies-Anne W.F.M. Severijnen, Andre Hoogeveen, Ben A. Oostra, Rob Willemsen
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引用次数: 33

摘要

FMR1(脆性X智力发育迟滞基因1)基因产物、蛋白FMRP(脆性X智力发育迟滞蛋白)的缺失导致脆性X综合征。FMRP与两个同源物FXR1P和FXR2P属于rna结合蛋白(FXR蛋白)的一个小家族。FXR蛋白的确切生理功能尚不清楚,但有人认为它在mRNA转运中起作用。在本研究中,我们在小鼠胚胎发育过程中对这些蛋白进行了免疫定位,以进一步了解它们的生理功能。这三种蛋白在小鼠胚胎发育过程中均有表达,但每种蛋白在不同发育阶段的表达模式和表达强度各不相同。在发育早期,Fxr蛋白的分布表现出高度的相似性,然而,在发育后期和新生儿中,观察到更多的差异表达,特别是在一些非神经组织中。这一描述性研究的结果讨论了脆性X综合征的发病机制。
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Immunocytochemical characterization of FMRP, FXR1P and FXR2P during embryonic development in the mouse

The absence of the FMR1 (fragile X mental retardation gene 1) gene product, protein FMRP (fragile X mental retardation protein) is causing the fragile X syndrome. FMRP, together with two homologues, called FXR1P and FXR2P, belongs to a small family of RNA-binding proteins (FXR proteins). The precise physiological function of the FXR proteins is unknown, but a role in mRNA transport has been suggested. In the present study, we have performed immunolocalization of these proteins during the embryonic development of the mouse to get more insight in their physiological function. All three proteins are expressed during mouse embryonic development, however, the pattern and intensity varies for each protein at the different developmental stages. During early development, the distribution of the Fxr proteins exhibits high similarities, however, during late development and in the neonate a more differential expression is observed especially in some non-neural tissues. The results of this descriptive study are discussed in relation to the pathogenesis of the fragile X syndrome.

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