Institute of Cell Biology and Neurobiology, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany; Lobachevsky State University of Nizhny Novgorod, Gagarina ave 23, 603950 Nizhny Novgorod, Russia.
Lobachevsky State University of Nizhny Novgorod, Gagarina ave 23, 603950 Nizhny Novgorod, Russia.
Brain Res. 2019 Feb 15;1705:24-31. doi: 10.1016/j.brainres.2018.09.034. Epub 2018 Sep 25.
Zeb2 (Sip1, Zfhx1b) is a transcription factor that plays essential role in neuronal development. Sip1 mutation in humans was shown to cause Mowat-Wilson syndrome, a syndromic form of Hirschprung's disease. Affected individuals exhibit multiple severe neurodevelopmental defects. Zeb2 can act as both transcriptional repressor and activator. It controls expression of a wide number of genes that regulate various aspects of neuronal development. This review addresses the molecular pathways acting downstream of Zeb2 that cause brain development disorders.
Zeb2（Sip1，Zfhx1b）是一种在神经元发育中起关键作用的转录因子。人类的 Sip1 突变被证实会导致 Mowat-Wilson 综合征，这是一种亨氏病的综合征形式。受影响的个体表现出多种严重的神经发育缺陷。Zeb2 可以作为转录抑制剂和激活剂。它控制着广泛数量的基因的表达，这些基因调节神经元发育的各个方面。这篇综述讨论了 Zeb2 下游作用的分子途径，这些途径导致了脑发育障碍。
