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
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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。
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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.

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来源期刊
遗传
遗传 Medicine-Medicine (all)
CiteScore
2.50
自引率
0.00%
发文量
6699
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