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在自闭症谱系障碍的遗传模型中研究儿童发育。

Studying child development in genetic models of ASD.

作者信息

Garg Shruti, Green Jonathan

机构信息

Division of Neuroscience & Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, United Kingdom; Manchester Academic Health Science Centre, Manchester University NHS Foundation Trust, Greater Manchester Mental Health NHS Trust, Manchester, United Kingdom.

Division of Neuroscience & Experimental Psychology, School of Biological Sciences, University of Manchester, Manchester, United Kingdom; Manchester Academic Health Science Centre, Manchester University NHS Foundation Trust, Greater Manchester Mental Health NHS Trust, Manchester, United Kingdom.

出版信息

Prog Brain Res. 2018;241:159-192. doi: 10.1016/bs.pbr.2018.09.009. Epub 2018 Nov 1.

Abstract

This chapter approaches the early development in autism spectrum disorder (ASD) through comparative study of some key monogenic syndromic models of ASD in humans. Using this method, as well as referring to relevant work in idiopathic ASD, we address three complimentary areas: (i) patterns of ASD behavioral phenotype expression across genetic syndromes, as a way of addressing gene-phenotype correlations; (ii) longitudinal developmental trajectories toward autism in early childhood, as a way of addressing developmental specificity; and (iii) experimental intervention trials, for treatment and mechanism discovery. The comparative approach does not highlight striking phenotypic specificity, but early studies were often limited and more methodologically sophisticated recent studies may suggest subtle distinctions. Longitudinal studies are at an early stage but can build on the substantive work on early prodromal development of idiopathic ASD. Translational intervention trials to date have not found candidate treatments and we argue that a new generation of more ambitious experimental mechanism trials is needed. This field now has the opportunity to combine comparative prospective longitudinal developmental studies with in-depth cross-syndrome phenotyping and linked ambitious targeted mechanistic interventions in a way that could be mutually informing and maximize the potential of syndromic models to illuminate the pathophysiology of ASD.

摘要

本章通过对人类自闭症谱系障碍(ASD)一些关键单基因综合征模型的比较研究,探讨ASD的早期发展。运用这种方法,并参考特发性ASD的相关研究工作,我们探讨三个互补领域:(i)跨遗传综合征的ASD行为表型表达模式,以此探讨基因-表型相关性;(ii)幼儿期向自闭症发展的纵向发育轨迹,以此探讨发育特异性;(iii)用于治疗和机制发现的实验性干预试验。比较方法并未突出显著的表型特异性,但早期研究往往存在局限性,而近期方法更复杂的研究可能会揭示细微差异。纵向研究尚处于早期阶段,但可以基于特发性ASD早期前驱期发展的大量实质性工作。迄今为止,转化性干预试验尚未找到候选治疗方法,我们认为需要新一代更具雄心的实验性机制试验。该领域现在有机会将比较性前瞻性纵向发育研究与深入的跨综合征表型分析以及相关的雄心勃勃的靶向机制性干预相结合,以相互提供信息,并最大限度地发挥综合征模型阐明ASD病理生理学的潜力。

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