Raefski Adam S, Carone Benjamin R, Kaur Anupinder, Krueger Winfried, O'Neill Michael J
Department of Molecular and Cell Biology, University of Connecticut, Storrs, CT 06269, USA.
J Mol Neurosci. 2007;32(2):111-9. doi: 10.1007/s12031-007-0022-7.
Certain neurobehavioral deficiencies associated with Turner Syndrome have been attributed to brain volumetric abnormalities, particularly of the amygdala. Haplo-insufficiency of a non-dosage compensated gene or genes on the X chromosome has been hypothesized to be the cause of the neuroanatomical defect. We examined gene expression levels of 6,628 genes in developing amygdalae of late-stage embryos of a mouse model for Turner Syndrome. In total, 161 genes show significant differences in expression level between TS and normal female amygdala. In silico pathway analysis of both X-linked and autosomal mis-regulated genes suggests that modulation of Wnt signaling is a critical factor in the normal growth and development of the amygdala.
某些与特纳综合征相关的神经行为缺陷被认为与脑容量异常有关，尤其是杏仁核的异常。据推测，X染色体上一个或多个非剂量补偿基因的单倍体不足是神经解剖学缺陷的原因。我们检测了特纳综合征小鼠模型晚期胚胎发育中的杏仁核中6628个基因的表达水平。总体而言，161个基因在特纳综合征和正常雌性杏仁核之间的表达水平存在显著差异。对X连锁和常染色体失调基因的计算机通路分析表明，Wnt信号通路的调节是杏仁核正常生长和发育的关键因素。
