Li Yong, Cheng Qi, Qian Jian-hua, Zhou Wei, Xie Xing, Slim Rima
Department of Obstetrics and Gynaecology, Women's Hospital, School of Medicine, Zhejiang University, Zhejiang 310006, China.
Zhonghua Fu Chan Ke Za Zhi. 2011 Aug;46(8):600-4.
To evaluate the NLRP7 gene mutations and variants and their expression of genetic approach in hydatidiform mole patients with family history.
Six cases of mole patients with family members of mole history and 60 healthy women, taking blood, extracting DNA, the genetic mutation on NLRP7 screening and analysis, looking for mutations and corresponding amino acids, proteins control gene mutation found NLRP7 area.
In 6 mole patients with family history: three patients were with sister's history of mole, and 2 of them familial recurrent hydatidiform mole (from family MoCh76 and family Ch77), there are 2 loci NLRP7 gene mutation. Screening patients from family MoCh76 for mutations in NLRP7 revealed in exon 3 and exon 5, amino acids [295G > T] and [1970A > T], proteins [Glu99X] and [Asp657Val], in a heterozygous. Screening patients from family Ch77 for mutations in NLRP7 revealed in exon 4 and exon 7, amino acids [1294C > T] and [2471 + 1G > A], proteins [Arg432X] and [Leu825X], in a heterozygous. Screening patients from family 105 for mutations in NLRP7 revealed no NLRP7 gene mutation. There were mother's history of mole in three patients, and they were not familial recurrent hydatidiform mole. Screening patients from family MoCh73 for mutations in NLRP7 revealed in exon 4, amino acids [1137G > C], proteins [Lys379Asn], in a heterozygous. Screening patients from family 106 and family 110 for mutations in NLRP7 revealed no NLRP7 gene mutation. There were not found mutations and variations in 60 cases of ethnic matched control group.
NLRP7 mutations may be lead to familial recurrent hydatidiform mole.
评估有家族史的葡萄胎患者中NLRP7基因突变及变异情况及其遗传方式的表达。
选取6例有葡萄胎家族史的葡萄胎患者及60例健康女性,采集血液,提取DNA,对NLRP7进行基因突变筛查及分析,寻找突变位点及相应氨基酸、蛋白质,检测NLRP7基因控制区基因突变情况。
在6例有家族史的葡萄胎患者中:3例患者有姐妹患葡萄胎史,其中2例为家族性复发性葡萄胎(来自MoCh76家族和Ch77家族),NLRP7基因有2个位点突变。对MoCh76家族患者进行NLRP7基因突变筛查,在外显子3和外显子5发现氨基酸[295G>T]和[1970A>T],蛋白质[Glu99X]和[Asp657Val],呈杂合状态。对Ch77家族患者进行NLRP7基因突变筛查,在外显子4和外显子7发现氨基酸[1294C>T]和[2471+1G>A],蛋白质[Arg432X]和[Leu825X],呈杂合状态。对105家族患者进行NLRP7基因突变筛查,未发现NLRP7基因突变。3例患者有母亲患葡萄胎史,且非家族性复发性葡萄胎。对MoCh73家族患者进行NLRP7基因突变筛查,在外显子4发现氨基酸[1137G>C],蛋白质[Lys379Asn],呈杂合状态。对106家族和110家族患者进行NLRP7基因突变筛查,未发现NLRP7基因突变。在60例种族匹配的对照组中未发现突变及变异。
NLRP7突变可能导致家族性复发性葡萄胎。