肠道神经系统条纹图案与疾病:尚未探索的病理生理学。

IF 7.1 1区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY Cellular and Molecular Gastroenterology and Hepatology Pub Date : 2024-01-01 DOI:10.1016/j.jcmgh.2024.03.004
Lori B. Dershowitz , Julia A. Kaltschmidt
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引用次数: 0

摘要

肠神经系统(ENS)控制着胃肠道(GI)的运动,ENS发育缺陷是小儿胃肠道运动障碍的基础。在赫氏普隆氏病(HSCR)、小儿肠假性梗阻(PIPO)和 B 型肠神经元发育不良(INDB)等疾病中,ENS 结构发生了改变,赫氏普隆氏病的神经元密度明显下降,而 PIPO 和 INDB 的神经元密度则有增加的报道。这些结构缺陷的发育起源尚未完全明了。在此,我们结合有关耳鼻咽喉神经系统结构的新数据,回顾了目前对耳鼻咽喉神经系统发育和小儿消化道运动障碍的认识。特别是,新出现的证据表明,在小鼠和人类的发育过程中,肠神经元沿着肠道纵轴呈环形条纹状分布。对 ENS 结构的这一新认识为儿科消化道运动障碍的病理生理学提出了新的问题。如果 ENS 被组织成条纹状,那么在 HSCR、PIPO 和 INDB 中观察到的肠神经元密度变化是否代表肠神经元条纹分布的差异?在此,我们回顾了其他生物系统的条纹图案化机制,并提出条纹状 ENS 图案化缺陷如何解释在小儿消化道运动障碍中观察到的结构缺陷。
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Enteric Nervous System Striped Patterning and Disease: Unexplored Pathophysiology

The enteric nervous system (ENS) controls gastrointestinal (GI) motility, and defects in ENS development underlie pediatric GI motility disorders. In disorders such as Hirschsprung’s disease (HSCR), pediatric intestinal pseudo-obstruction (PIPO), and intestinal neuronal dysplasia type B (INDB), ENS structure is altered with noted decreased neuronal density in HSCR and reports of increased neuronal density in PIPO and INDB. The developmental origin of these structural deficits is not fully understood. Here, we review the current understanding of ENS development and pediatric GI motility disorders incorporating new data on ENS structure. In particular, emerging evidence demonstrates that enteric neurons are patterned into circumferential stripes along the longitudinal axis of the intestine during mouse and human development. This novel understanding of ENS structure proposes new questions about the pathophysiology of pediatric GI motility disorders. If the ENS is organized into stripes, could the observed changes in enteric neuron density in HSCR, PIPO, and INDB represent differences in the distribution of enteric neuronal stripes? We review mechanisms of striped patterning from other biological systems and propose how defects in striped ENS patterning could explain structural deficits observed in pediatric GI motility disorders.

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来源期刊
CiteScore
13.00
自引率
2.80%
发文量
246
审稿时长
42 days
期刊介绍: "Cell and Molecular Gastroenterology and Hepatology (CMGH)" is a journal dedicated to advancing the understanding of digestive biology through impactful research that spans the spectrum of normal gastrointestinal, hepatic, and pancreatic functions, as well as their pathologies. The journal's mission is to publish high-quality, hypothesis-driven studies that offer mechanistic novelty and are methodologically robust, covering a wide range of themes in gastroenterology, hepatology, and pancreatology. CMGH reports on the latest scientific advances in cell biology, immunology, physiology, microbiology, genetics, and neurobiology related to gastrointestinal, hepatobiliary, and pancreatic health and disease. The research published in CMGH is designed to address significant questions in the field, utilizing a variety of experimental approaches, including in vitro models, patient-derived tissues or cells, and animal models. This multifaceted approach enables the journal to contribute to both fundamental discoveries and their translation into clinical applications, ultimately aiming to improve patient care and treatment outcomes in digestive health.
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