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人类疾病的非遗传性遗传学:聚焦于个体一生中获得的合子后遗传变异。

Non-heritable genetics of human disease: spotlight on post-zygotic genetic variation acquired during lifetime.

机构信息

Department of Immunology, Genetics and Pathology, Rudbeck Laboratory, Uppsala University, Uppsala, Sweden.

出版信息

J Med Genet. 2013 Jan;50(1):1-10. doi: 10.1136/jmedgenet-2012-101322. Epub 2012 Nov 21.

Abstract

The heritability of most common, multifactorial diseases is rather modest and known genetic effects account for a small part of it. The remaining portion of disease aetiology has been conventionally ascribed to environmental effects, with an unknown part being stochastic. This review focuses on recent studies highlighting stochastic events of potentially great importance in human disease-the accumulation of post-zygotic structural aberrations with age in phenotypically normal humans. These findings are in agreement with a substantial mutational load predicted to occur during lifetime within the human soma. A major consequence of these results is that the genetic profile of a single tissue collected at one time point should be used with caution as a faithful portrait of other tissues from the same subject or the same tissue throughout life. Thus, the design of studies in human genetics interrogating a single sample per subject or applying lymphoblastoid cell lines may come into question. Sporadic disorders are common in medicine. We wish to stress the non-heritable genetic variation as a potentially important factor behind the development of sporadic diseases. Moreover, associations between post-zygotic mutations, clonal cell expansions and their relation to cancer predisposition are central in this context. Post-zygotic mutations are amenable to robust examination and are likely to explain a sizable part of non-heritable disease causality, which has routinely been thought of as synonymous with environmental factors. In view of the widespread accumulation of genetic aberrations with age and strong predictions of disease risk from such analyses, studies of post-zygotic mutations may be a fruitful approach for delineation of variants that are causative for common human disorders.

摘要

大多数常见的多因素疾病的遗传性相当适中,已知的遗传效应只占其中的一小部分。疾病病因的剩余部分传统上归因于环境影响,其中未知的部分是随机的。这篇综述重点介绍了最近的研究结果,这些结果强调了随机事件在人类疾病中的潜在重要性——在表型正常的人类中,随着年龄的增长,合子后结构异常的积累。这些发现与在人类躯体一生中预测会发生大量突变负荷的观点一致。这些结果的一个主要后果是,在同一时间点收集的单个组织的遗传谱应该谨慎使用,因为它不能准确反映同一受试者的其他组织或整个生命周期中的同一组织的遗传谱。因此,对每个受试者进行一次单一样本的人类遗传学研究设计或应用淋巴母细胞系可能会受到质疑。散发性疾病在医学中很常见。我们希望强调非遗传性遗传变异可能是散发性疾病发展背后的一个重要因素。此外,合子后突变、克隆细胞扩增及其与癌症易感性的关系在这方面是核心。合子后突变易于进行稳健的检查,并且很可能解释了相当一部分不可遗传疾病的病因,这些病因通常被认为与环境因素同义。鉴于随着年龄的增长遗传异常的广泛积累以及此类分析对疾病风险的强烈预测,合子后突变的研究可能是确定导致常见人类疾病的变异的一种富有成效的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad7d/3534255/13f0a2223dc2/jmedgenet-2012-101322f01.jpg

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