近在咫尺却又遥不可及:基因印记疾病的管理策略研究。

IF 1.5 Q4 CELL BIOLOGY American journal of stem cells Pub Date : 2018-10-01 eCollection Date: 2018-01-01
Mark A Pianka, Alec T McIntosh, Sahaj D Patel, Pegah R Bakhshi, Mira Jung
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引用次数: 0

摘要

遗传印记是根据基因的母系或父系起源,以遗传模式对特定基因进行表观遗传标记或沉默的过程。由于这些先天性综合征与辅助生殖技术(ARTs)之间的潜在关联,印迹疾病的发生率日益受到关注。本文综述了印迹过程的总体概况,以及目前关于最常见的印迹疾病的遗传和表观遗传基础的知识:beckwithi - wiedemann综合征(BWS)、Silver-Russell综合征(SRS)、Prader-Willi综合征(PWS)和Angelman综合征(as)。随着研究继续阐明表征遗传印记的分子途径,人们努力建立结合表型表现和基因检测的指导方针,以确保安全有效地管理症状。虽然这些努力可能有利于未来的临床管理,但它们的功效还不能推广到所有被诊断患有这些综合征的患者,因为许多遗传异常和相关的表型表现尚未被表征。此外,在表观遗传过程和基因位点的知识参与这些综合征的发展的未来进展可能允许治疗的发展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Close yet so far away: a look into the management strategies of genetic imprinting disorders.

Genetic imprinting is the process of epigenetic labelling or silencing of particular genes, based on the maternal or paternal origin of the gene, in a heritable pattern. The incidence of imprinting disorders has become a growing concern due to the potential association between these congenital syndromes and assisted reproductive technologies (ARTs). This review presents a general summary of the imprinting process as well as the current knowledge surrounding the genetic and epigenetic underpinnings of the most prevalent imprinting disorders: Beckwith-Wiedemann syndrome (BWS), Silver-Russell syndrome (SRS), Prader-Willi syndrome (PWS), and Angelman syndrome (AS). As research continues to elucidate the molecular pathways that characterize genetic imprinting, efforts have been made to establish guidelines that incorporate phenotypic manifestations as well as genetic testing to ensure safe and effective management of symptoms. While these efforts are likely to benefit future clinical management, their efficacy cannot yet be generalized to all patients diagnosed with these syndromes, as many of the genetic abnormalities and the associated phenotypic manifestations have yet to be characterized. Furthermore, future advances in the knowledge of epigenetic processes and genetic loci involved in the development of these syndromes may allow for the development of curative therapies.

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