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遗传学中基于RNA的诊断研究:来自法国多学科网络的综述与指南

RNA-based diagnostic studies in genetics: Review and guidance from a multidisciplinary French network.

作者信息

Buisine Marie-Pierre, Bellanne-Chantelot Christine, Calmels Nadège, Vaché Christel, Besnard Thomas, Cogne Benjamin, Vitobello Antonio, Piton Amélie, Martins Alexandra, Gaildrat Pascaline, Dhaenens Claire-Marie, Gorokhova Svetlana, Boutry-Kryza Nadia, Caputo Sandrine, Leman Raphaël, Krieger Sophie, Le Gac Gérald, Houdayer Claude

机构信息

Univ. Lille, CNRS, Inserm, CHU Lille, UMR9020-U1277 CANTHER - Cancer Heterogeneity Plasticity and Resistance to Therapies, F-59000, Lille, France.

AP-HP. Sorbonne University, Pitié-Salpêtrière Hospital, Department of Medical Genetics, F-75013, Paris, France.

出版信息

Eur J Hum Genet. 2025 Jun 5. doi: 10.1038/s41431-025-01881-2.

Abstract

The widespread use of high-throughput sequencing for genetic diagnosis has led to considerable advances in patient care, but interpretation of the variants identified remains a challenge and geneticists routinely face the question of variants of uncertain significance. The clinical interpretation of genomic variants requires a high level of expertise to ensure appropriate genetic counseling. Assessing the impact of variants on splicing is a key issue in order to determine their pathogenicity as each variant can impact pre-mRNA splicing by disruption of the splicing code. It is for this reason that a diverse group of French molecular and clinical genetics experts from different diagnostic laboratories nationwide was established to discuss splicing issues and elaborate diagnostic recommendations. We describe an update of these recommendations with the aim of highlighting the importance of transcript characterization for variant interpretation and facilitating the diagnostic implementation of transcript studies, an important source of new diagnostics in human genetics.

摘要

高通量测序在基因诊断中的广泛应用已在患者护理方面取得了相当大的进展,但对所鉴定变异的解读仍然是一项挑战,遗传学家经常面临意义未明变异的问题。基因组变异的临床解读需要高水平的专业知识以确保进行适当的遗传咨询。评估变异对剪接的影响是确定其致病性的关键问题,因为每个变异都可能通过破坏剪接密码来影响前体mRNA剪接。正是出于这个原因,来自全国不同诊断实验室的一群法国分子和临床遗传学专家成立了,以讨论剪接问题并制定诊断建议。我们描述了这些建议的更新,旨在强调转录本特征对于变异解读的重要性,并促进转录本研究在诊断中的应用,转录本研究是人类遗传学新诊断方法的一个重要来源。

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