Frullanti Elisa, Amabile Sonia, Lolli Maria Grazia, Bartolini Anna, Livide Gabriella, Landucci Elisa, Mari Francesca, Vaccarino Flora M, Ariani Francesca, Massimino Luca, Renieri Alessandra, Meloni Ilaria
Genetica Medica, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Medical Genetics, University of Siena, Siena, Italy.
Eur J Hum Genet. 2016 Feb;24(2):252-7. doi: 10.1038/ejhg.2015.79. Epub 2015 May 13.
Foxg1 gene encodes for a transcription factor essential for telencephalon development in the embryonic mammalian forebrain. Its complete absence is embryonic lethal while Foxg1 heterozygous mice are viable but display microcephaly, altered hippocampal neurogenesis and behavioral and cognitive deficiencies. In order to evaluate the effects of Foxg1 alteration in adult brain, we performed expression profiling in total brains from Foxg1+/- heterozygous mutants and wild-type littermates. We identified statistically significant differences in expression levels for 466 transcripts (P<0.001), 29 of which showed a fold change ≥ 1.5. Among the differentially expressed genes was found a group of genes expressed in the basal ganglia and involved in the control of movements. A relevant (three to sevenfold changes) and statistically significant increase of expression, confirmed by qRT-PCR, was found in two highly correlated genes with expression restricted to the hypothalamus: Oxytocin (Oxt) and Arginine vasopressin (Avp). These neuropeptides have an important role in maternal and social behavior, and their alteration is associated with impaired social interaction and autistic behavior. In addition, Neuronatin (Nnat) levels appear significantly higher both in Foxg1+/- whole brain and in hippocampal neurons after silencing Foxg1, strongly suggesting that it is directly or indirectly repressed by Foxg1. During fetal and neonatal brain development, Nnat may regulate neuronal excitability, receptor trafficking and calcium-dependent signaling and, in the adult brain, it is predominantly expressed in parvalbumin-positive GABAergic interneurons. Overall, these results implicate the overexpression of a group of neuropeptides in the basal ganglia, hypothalamus, cortex and hippocampus in the pathogenesis FOXG1 behavioral impairments.
Foxg1基因编码一种转录因子,该转录因子对胚胎哺乳动物前脑端脑的发育至关重要。完全缺失该基因会导致胚胎致死,而Foxg1杂合小鼠可以存活,但表现出小头畸形、海马神经发生改变以及行为和认知缺陷。为了评估Foxg1改变对成体大脑的影响,我们对Foxg1+/-杂合突变体和野生型同窝小鼠的全脑进行了表达谱分析。我们确定了466个转录本的表达水平存在统计学显著差异(P<0.001),其中29个转录本的变化倍数≥1.5。在差异表达基因中,发现了一组在基底神经节表达且参与运动控制的基因。通过qRT-PCR证实,在两个高度相关且表达局限于下丘脑的基因中发现了显著(三到七倍变化)且具有统计学意义的表达增加:催产素(Oxt)和精氨酸加压素(Avp)。这些神经肽在母性行为和社会行为中具有重要作用,其改变与社交互动受损和自闭症行为有关。此外,在Foxg1+/-全脑以及沉默Foxg1后的海马神经元中,神经调蛋白(Nnat)水平均显著升高,强烈表明它直接或间接受Foxg1抑制。在胎儿和新生儿大脑发育过程中,Nnat可能调节神经元兴奋性、受体转运和钙依赖性信号传导,而在成体大脑中,它主要在小白蛋白阳性的GABA能中间神经元中表达。总体而言,这些结果表明基底神经节、下丘脑、皮层和海马中一组神经肽的过表达与FOXG1行为障碍的发病机制有关。