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首页> 外文期刊>Human Molecular Genetics >Filamin A and Filamin B are co-expressed within neurons during periods of neuronal migration and can physically interact.
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Filamin A and Filamin B are co-expressed within neurons during periods of neuronal migration and can physically interact.

机译：Filamin A和Filamin B在神经元迁移期间在神经元内共表达，并且可以发生物理相互作用。

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摘要

Mutations in the X-linked gene Filamin A (FLNA) lead to the human neurological disorder, periventricular heterotopia (PH). Although PH is characterized by a failure in neuronal migration into the cerebral cortex with consequent formation of nodules in the ventricular and subventricular zones, many neurons appear to migrate normally, even in males, suggesting compensatory mechanisms. Here we characterize expression patterns for FlnA and a highly homologous protein Filamin B (FlnB) within the nervous system, in order to better understand their potential roles in cortical development. FlnA mRNA was widely expressed in all cortical layers while FlnB mRNA was most highly expressed in the ventricular and subventricular zones during development. In adulthood, widespread but reduced expression of FlnA and FlnB persisted throughout the cerebral cortex. FlnA and FlnB proteins were highly expressed in both the leading processes and somata of migratory neurons during corticogenesis. Postnatally, FlnA immunoreactivity was largely localized to the cell body with FlnB in the soma and neuropil during neuronal differentiation. In adulthood, diminished expression of both proteins localized to the cell soma and nucleus. Moreover, the putative FLNB homodimerization domain strongly interacted with itself or the corresponding homologous region of FLNA by yeast two-hybrid interaction, the two proteins co-localized within neuronal precursors by immunocytochemistry and the existence of FLNA-FLNB heterodimers could be detected by co-immunoprecipitation. These results suggest that FLNA and FLNB may form both homodimers and heterodimers and that their interaction could potentially compensate for the loss of FLNA function during cortical development within PH individuals.
机译：X连锁基因Filamin A（FLNA）的突变会导致人类神经系统疾病，即脑室周围异位症（PH）。尽管PH的特征是神经元迁移到大脑皮层失败，并在心室和脑室下区域形成结节，但许多神经元似乎正常迁移，甚至在男性中也是如此，这表明存在补偿机制。在这里，我们表征神经系统内FlnA和高度同源的蛋白质Filamin B（FlnB）的表达模式，以便更好地了解它们在皮层发育中的潜在作用。 FlnA mRNA在所有皮层中广泛表达，而FlnB mRNA在发育过程中在心室和心室下区域表达最高。在成年期，遍及整个大脑皮层的FlnA和FlnB表达普遍但降低。 FlnA和FlnB蛋白在皮质发生过程中在迁移神经元的前导过程和躯体中高表达。出生后，在神经元分化过程中，FlnA的免疫反应性主要定位于细胞体中的FlnB在体细胞和神经纤维中。成年后，位于细胞体和细胞核的两种蛋白质的表达均降低。此外，推定的FLNB同源二聚结构域通过酵母双杂交相互作用与其自身或相应的FLNA同源区域强烈相互作用，两种蛋白通过免疫细胞化学共定位在神经元前体中，并且FLNA-FLNB异二聚体的存在可以通过共免疫沉淀。这些结果表明，FLNA和FLNB可能同时形成同二聚体和异二聚体，它们的相互作用可能会补偿PH个体在皮质发育过程中FLNA功能的丧失。

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