Monuki Edwin S
Department of Pathology and Laboratory Medicine, UC Irvine School of Medicine, University of California-Irvine, Irvine, CA 92697, USA.
J Neuropathol Exp Neurol. 2007 Jul;66(7):566-75. doi: 10.1097/nen.0b013e3180986e1b.
Forebrain development is directed by secreted signaling molecules known as morphogens, and morphogen signaling defects often lead to failed midline induction and holoprosencephaly (HPE), the most common malformation of the human forebrain. Genetic studies in multiple organisms implicate 4 well-known morphogens or morphogen families--Nodal, Sonic hedgehog, Fibroblast growth factors, and Bone morphogenetic proteins--as causes of HPE. Here I review the roles of these morphogens in HPE and forebrain midline development. In particular, this review focuses on recent evidence for cross-regulatory interactions between morphogens, which lead to a signaling network model of forebrain development that can explain the distinctive HPE phenotypes seen in humans and animal models.
前脑发育由被称为形态发生素的分泌信号分子所引导，形态发生素信号缺陷常常导致中线诱导失败和全前脑畸形（HPE），这是人类前脑最常见的畸形。对多种生物体的遗传学研究表明，4种著名的形态发生素或形态发生素家族——Nodal、音猬因子、成纤维细胞生长因子和骨形态发生蛋白——是导致HPE的原因。在此，我综述这些形态发生素在HPE和前脑中线发育中的作用。特别地，本综述聚焦于形态发生素之间交叉调节相互作用的最新证据，这些证据形成了一个前脑发育的信号网络模型，该模型能够解释在人类和动物模型中所见的独特HPE表型。
