模型系统在理解人类神经病变细胞信号复杂性中的关键作用。

IF 4.6 3区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL
WIREs Mechanisms of Disease Pub Date : 2022-01-01 Epub Date: 2021-10-13 DOI:10.1002/wsbm.1537
Santiago Cerrizuela, Guillermo A Vega-Lopez, Karla Méndez-Maldonado, Iván Velasco, Manuel J Aybar
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引用次数: 3

摘要

动物模型有助于研究正常和病理发育的分子、细胞和形态发生机制。基于细胞的研究模型已经成为研究人类胚胎发育和疾病的许多方面的一种替代方法。神经嵴(NC)是一种短暂的、多能的、迁移的胚胎细胞群,在脊椎动物发育过程中产生多种细胞类型。NC的异常形成或发育导致神经嵴病变(ncp),其特征是广泛的功能和形态改变。导致这些多表型疾病的受损分子机制尚不完全清楚。这一事实,再加上这些疾病在新生儿中的高发病率,导致了对其理解的系统方法的发展。在这篇文章中,我们系统地回顾了不同动物和细胞模型系统的实验如何提高了我们对ncp的认识,以及这些进展如何有助于开发更好的诊断和治疗工具来治疗这些病理。本文分类如下:先天性疾病>遗传学/基因组学/表观遗传学先天性疾病>干细胞和发育先天性疾病>分子和细胞生理学神经疾病>遗传学/基因组学/表观遗传学。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The crucial role of model systems in understanding the complexity of cell signaling in human neurocristopathies.

Animal models are useful to study the molecular, cellular, and morphogenetic mechanisms underlying normal and pathological development. Cell-based study models have emerged as an alternative approach to study many aspects of human embryonic development and disease. The neural crest (NC) is a transient, multipotent, and migratory embryonic cell population that generates a diverse group of cell types that arises during vertebrate development. The abnormal formation or development of the NC results in neurocristopathies (NCPs), which are characterized by a broad spectrum of functional and morphological alterations. The impaired molecular mechanisms that give rise to these multiphenotypic diseases are not entirely clear yet. This fact, added to the high incidence of these disorders in the newborn population, has led to the development of systematic approaches for their understanding. In this article, we have systematically reviewed the ways in which experimentation with different animal and cell model systems has improved our knowledge of NCPs, and how these advances might contribute to the development of better diagnostic and therapeutic tools for the treatment of these pathologies. This article is categorized under: Congenital Diseases > Genetics/Genomics/Epigenetics Congenital Diseases > Stem Cells and Development Congenital Diseases > Molecular and Cellular Physiology Neurological Diseases > Genetics/Genomics/Epigenetics.

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来源期刊
WIREs Mechanisms of Disease
WIREs Mechanisms of Disease MEDICINE, RESEARCH & EXPERIMENTAL-
CiteScore
11.40
自引率
0.00%
发文量
45
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