Building a brain in the gut: development of the enteric nervous system.

Building a brain in the gut: development of the enteric nervous system.
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在肠道中建立大脑:肠神经系统的发展。

DOI:
10.1111/cge.12054
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发表时间:
2013-04
期刊:
影响因子:
3.5
通讯作者:
Burns AJ
Burns AJ
中科院分区:
医学2区
文献类型:
--
作者:
Goldstein AM;Hofstra RM;Burns AJ

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肠神经系统(ENS)是胃肠道的内在神经支配,是肠道神经肌肉组织的重要组成部分,控制着肠道功能的许多方面,包括协调的肌肉扩张。ENS完全来源于神经嵴细胞(NCC),其在胚胎发生和胎儿生命期间经历许多关键过程,包括广泛迁移到肠道中并沿着肠道、增殖和分化成肠神经元和神经胶质。这些机制受到多种信号通路、转录因子、神经营养因子和细胞外基质成分的分子控制。这些过程的失败和随后的异常ENS发育可导致所谓的肠神经病,可以说其中最具特征的是先天性疾病先天性巨结肠(HSCR)或无神经节巨结肠。这篇综述主要集中在调节ENS从NCC发展的分子和遗传因素,HSCR及其相关综合征的临床遗传学,以及旨在提高我们对肠神经病的理解和治疗的最新进展。
The enteric nervous system (ENS), the intrinsic innervation of the gastrointestinal tract, is an essential component of the gut neuromusculature and controls many aspects of gut function, including coordinated muscular peristalsis. The ENS is entirely derived from neural crest cells (NCC) which undergo a number of key processes, including extensive migration into and along the gut, proliferation, and differentiation into enteric neurons and glia, during embryogenesis and fetal life. These mechanisms are under the molecular control of numerous signaling pathways, transcription factors, neurotrophic factors and extracellular matrix components. Failure in these processes and consequent abnormal ENS development can result in so-called enteric neuropathies, arguably the best characterized of which is the congenital disorder Hirschsprung disease (HSCR), or aganglionic megacolon. This review focuses on the molecular and genetic factors regulating ENS development from NCC, the clinical genetics of HSCR and its associated syndromes, and recent advances aimed at improving our understanding and treatment of enteric neuropathies.
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