为一个患有手足分离畸形的中国家庭鉴定致病变体并进行植入前基因检测。

Q3 Medicine
遗传 Pub Date : 2024-09-01 DOI:10.16288/j.yczz.24-141
Li-Bin Mei, Yi-Yuan Zhang, Xian-Jing Huang, Hong Ji, Ping-Ping Qiu, Lu Ding, Xuemei He, Ping Li
{"title":"为一个患有手足分离畸形的中国家庭鉴定致病变体并进行植入前基因检测。","authors":"Li-Bin Mei, Yi-Yuan Zhang, Xian-Jing Huang, Hong Ji, Ping-Ping Qiu, Lu Ding, Xuemei He, Ping Li","doi":"10.16288/j.yczz.24-141","DOIUrl":null,"url":null,"abstract":"<p><p>Split-hand/foot malformation is a serious congenital limb malformation characterized by syndactyly and underdevelopment of the phalanges and metatarsals. In this study, we reported a case of a fetus with hand-foot cleft deformity. Whole exome and Sanger sequencing were used to filter out candidate gene mutation sites and provide pre-implantation genetic testing(PGT) for family members. Genetic testing results showed that there was a homozygous mutation c.786G>A (p.Trp262*) in the fetal <i>WNT10B</i>, and both parents were carriers of heterozygous mutations. PGT results showed that out of the two blastocysts, one was a heterozygous mutant and the other was a homozygous mutant. All the embryos had diploid chromosomes. The heterozygous embryo was transferred, and a singleton pregnancy was successfully achieved. This study suggests that homozygous mutations in <i>WNT10B</i> are the likely cause of hand-foot clefts in this family. For families with monogenic diseases, preimplantation genetic testing can effectively prevent the birth of an affected child only after identifying the pathogenic mutation.</p>","PeriodicalId":35536,"journal":{"name":"遗传","volume":"46 9","pages":"750-756"},"PeriodicalIF":0.0000,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Identification of a pathogenic variant and pre-implantation genetic testing for a Chinese family affected with split-hand/foot malformation.\",\"authors\":\"Li-Bin Mei, Yi-Yuan Zhang, Xian-Jing Huang, Hong Ji, Ping-Ping Qiu, Lu Ding, Xuemei He, Ping Li\",\"doi\":\"10.16288/j.yczz.24-141\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Split-hand/foot malformation is a serious congenital limb malformation characterized by syndactyly and underdevelopment of the phalanges and metatarsals. In this study, we reported a case of a fetus with hand-foot cleft deformity. Whole exome and Sanger sequencing were used to filter out candidate gene mutation sites and provide pre-implantation genetic testing(PGT) for family members. Genetic testing results showed that there was a homozygous mutation c.786G>A (p.Trp262*) in the fetal <i>WNT10B</i>, and both parents were carriers of heterozygous mutations. PGT results showed that out of the two blastocysts, one was a heterozygous mutant and the other was a homozygous mutant. All the embryos had diploid chromosomes. The heterozygous embryo was transferred, and a singleton pregnancy was successfully achieved. This study suggests that homozygous mutations in <i>WNT10B</i> are the likely cause of hand-foot clefts in this family. For families with monogenic diseases, preimplantation genetic testing can effectively prevent the birth of an affected child only after identifying the pathogenic mutation.</p>\",\"PeriodicalId\":35536,\"journal\":{\"name\":\"遗传\",\"volume\":\"46 9\",\"pages\":\"750-756\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"遗传\",\"FirstCategoryId\":\"1091\",\"ListUrlMain\":\"https://doi.org/10.16288/j.yczz.24-141\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"遗传","FirstCategoryId":"1091","ListUrlMain":"https://doi.org/10.16288/j.yczz.24-141","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0

摘要

手足裂畸形是一种严重的先天性肢体畸形,其特征是趾骨和跖骨联合actyly和发育不全。本研究报告了一例手足裂畸形胎儿。我们利用全外显子组测序和桑格测序筛选出候选基因突变位点,并为家庭成员提供植入前基因检测(PGT)。基因检测结果显示,胎儿的 WNT10B 存在一个 c.786G>A(p.Trp262*)的同基因突变,父母均为杂合突变携带者。PGT 结果显示,在两个囊胚中,一个是杂合突变体,另一个是同源突变体。所有胚胎都有二倍体染色体。杂合子胚胎移植后,成功实现了单胎妊娠。这项研究表明,WNT10B 的同源突变可能是导致该家族出现手足裂的原因。对于单基因遗传病家族,只有在确定致病突变后,植入前基因检测才能有效防止患儿的出生。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Identification of a pathogenic variant and pre-implantation genetic testing for a Chinese family affected with split-hand/foot malformation.

Split-hand/foot malformation is a serious congenital limb malformation characterized by syndactyly and underdevelopment of the phalanges and metatarsals. In this study, we reported a case of a fetus with hand-foot cleft deformity. Whole exome and Sanger sequencing were used to filter out candidate gene mutation sites and provide pre-implantation genetic testing(PGT) for family members. Genetic testing results showed that there was a homozygous mutation c.786G>A (p.Trp262*) in the fetal WNT10B, and both parents were carriers of heterozygous mutations. PGT results showed that out of the two blastocysts, one was a heterozygous mutant and the other was a homozygous mutant. All the embryos had diploid chromosomes. The heterozygous embryo was transferred, and a singleton pregnancy was successfully achieved. This study suggests that homozygous mutations in WNT10B are the likely cause of hand-foot clefts in this family. For families with monogenic diseases, preimplantation genetic testing can effectively prevent the birth of an affected child only after identifying the pathogenic mutation.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
遗传
遗传 Medicine-Medicine (all)
CiteScore
2.50
自引率
0.00%
发文量
6699
期刊介绍: Hereditas is a national academic journal sponsored by the Institute of Genetics and Developmental Biology of the Chinese Academy of Sciences and the Chinese Society of Genetics and published by Science Press. It is a Chinese core journal and a Chinese high-quality scientific journal. The journal mainly publishes innovative research papers in the fields of genetics, genomics, cell biology, developmental biology, biological evolution, genetic engineering and biotechnology; new technologies and new methods; monographs and reviews on hot issues in the discipline; academic debates and discussions; experience in genetics teaching; introductions to famous geneticists at home and abroad; genetic counseling; information on academic conferences at home and abroad, etc. Main columns: review, frontier focus, research report, technology and method, resources and platform, experimental operation guide, genetic resources, genetics teaching, scientific news, etc.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信