DMD/BMD植入前基因检测的十年应用:五种临床策略和胚胎重组模式分析。

IF 3.8 2区 生物学 Q2 GENETICS & HEREDITY
Weili Wang, Jing Dai, Xiao Hu, Wenbin He, Yifan Gu, Zhenxing Wan, Yi Zhang, Keli Luo, Wen Li, Qianjun Zhang, Fei Gong, Guangxiu Lu, Liang Hu, Yue-Qiu Tan, Ge Lin, Juan Du
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引用次数: 0

摘要

本研究旨在为Duchenne肌营养不良/Becker肌营养不良(DMD/BMD)患者寻找最有效的PGT-M治疗策略,减少DMD患者因胚胎重组引起的误诊。回顾性分析2009 - 2023年湘雅生殖遗传医院158个PGT-M周期DMD/BMD检测结果。收集患者的背景资料。我们分析了五种不同的PGT-M策略(1-5)的有效性和安全性,包括从卵裂细胞或育层外胚层(TE)细胞进行突变检测以及TE细胞扩增后的附加连锁分析。对这些周期的胚胎重组事件进行了评估。突变分析显示,125个家族中62.4%存在DMD缺失,16.0%存在重复,21.6%存在单核苷酸变异(snv)。125个家庭中,104个(83.2%)曾有胎儿或后代感染。将突变检测与基于snp的TE细胞连锁分析相结合的策略5的诊断率最高(99.56%)。这种策略在突变附近重组的情况下也显示出优势。胚胎的基因内重组率为5.5%,主要集中在DMD缺失/重复突变的热点区域(45-55和3-9外显子)。52个家庭的产前诊断和所有85例健康分娩的成功结果(活产率,65.89%,85/129)验证了PGT-M的准确性和有效性。本研究通过对5种不同策略的比较,提供了一种高效的DMD/BMD的PGT-M策略(策略5),诊断率达到99.56%。这些结果强调了监测基因内重组在DMD/BMD中常见的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Decade-long application of preimplantation genetic testing for DMD/BMD: analysis of five clinical strategies and embryo recombination patterns.

This study aimed to find the most effective PGT-M strategy for Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD), and to reduce misdiagnosis caused by embryo recombination in DMD. A retrospective study was performed by analyzing 158 PGT-M cycles for DMD/BMD in Reproductive and Genetic Hospital of CITIC-Xiangya between 2009 and 2023. Patients' backgrounds were collected. The effectiveness and safety for five different PGT-M strategies (1-5), including mutation testing from cleavage or trophoblast ectoderm (TE) cells and additional linkage analysis post-TE cell amplification, were analyzed. The embryonic recombination events were assessed for these cycles. Mutation analysis showed that 62.4% of the 125 families had DMD deletions, 16.0% had duplications, and 21.6% had single nucleotide variants (SNVs). Among 125 families, 104 (83.2%) had previously affected fetus or offspring. The highest diagnosis rate (99.56%) was achieved with Strategy 5, which combined mutation testing with SNP-based linkage analysis in TE cells. This strategy 5 also demonstrated an advantage in cases with recombination near the mutation. An intragenic recombination rate of 5.5% was observed in embryos, predominantly in the hotspots (exons 45-55 and exons 3-9) of DMD deletion/duplication mutations. Prenatal diagnosis for 52 families and successful outcomes in all 85 healthy deliveries (live birth rate, 65.89%, 85/129) validated the accuracy and effectiveness of PGT-M. This study provides a highly effective PGT-M strategy (Strategy 5) for DMD/BMD by comparing five different strategies, with the diagnostic yield reaching 99.56%. The results underscore the significance of monitoring intragenic recombination in DMD, which is a frequent occurrence in DMD/BMD.

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来源期刊
Human Genetics
Human Genetics 生物-遗传学
CiteScore
10.80
自引率
3.80%
发文量
94
审稿时长
1 months
期刊介绍: Human Genetics is a monthly journal publishing original and timely articles on all aspects of human genetics. The Journal particularly welcomes articles in the areas of Behavioral genetics, Bioinformatics, Cancer genetics and genomics, Cytogenetics, Developmental genetics, Disease association studies, Dysmorphology, ELSI (ethical, legal and social issues), Evolutionary genetics, Gene expression, Gene structure and organization, Genetics of complex diseases and epistatic interactions, Genetic epidemiology, Genome biology, Genome structure and organization, Genotype-phenotype relationships, Human Genomics, Immunogenetics and genomics, Linkage analysis and genetic mapping, Methods in Statistical Genetics, Molecular diagnostics, Mutation detection and analysis, Neurogenetics, Physical mapping and Population Genetics. Articles reporting animal models relevant to human biology or disease are also welcome. Preference will be given to those articles which address clinically relevant questions or which provide new insights into human biology. Unless reporting entirely novel and unusual aspects of a topic, clinical case reports, cytogenetic case reports, papers on descriptive population genetics, articles dealing with the frequency of polymorphisms or additional mutations within genes in which numerous lesions have already been described, and papers that report meta-analyses of previously published datasets will normally not be accepted. The Journal typically will not consider for publication manuscripts that report merely the isolation, map position, structure, and tissue expression profile of a gene of unknown function unless the gene is of particular interest or is a candidate gene involved in a human trait or disorder.
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