Department of Otolaryngology‑Head and Neck Surgery, Second Hospital of Lanzhou University, Lanzhou, Gansu 730030, P.R. China.
Mol Med Rep. 2017 Nov;16(5):6722-6728. doi: 10.3892/mmr.2017.7431. Epub 2017 Sep 7.
The present study aimed to investigate the molecular etiology of nonsyndromic hearing impairment (HI) in hearing impaired populations of Hui, Tibetan, and Tu ethnicities in northwest China. A total of 283 unrelated subjects with HI who attended special education schools in northwest China were enrolled in the present study. Single-nucleotide polymorphisms (SNPs) in three common deafness‑related genes, gap junction protein β2 (GJB2), solute carrier family 26 member 4 (SLC26A4) and mitochondrially encoded 12S RNA (mtDNA12SrRNA), were detected using a SNPscan technique. GJB2 mutations were detected in 14.89% of Hui patients, 9.37% of Tibetan patients and 11.83% of Tu patients. The most prevalent GJB2 mutation in the Hui and Tu patients was c.235delC. In the Tibetan patients, the c.109G>A SNP exhibited the highest allele frequency. SLC26A4 mutations were detected in 10.64% of Hui patients, 6.25% of Tibetan patients, and 8.6% of Tu patients. The most common SLC26A4 mutation was c.919‑2A>Gin the Hui, Tibetan, and Tu patients, and the second most common SLC26A4 mutations in these patients were c.1517T>G, c.1226G>A andc.2168A>G, respectively. The mutation rates ofmtDNA12SrRNA in the Hui, Tibetan, and Tu patients were 1.06, 5.21, and 5.38%, respectively. These findings demonstrate that the mutation spectra of these deafness‑related genes are unique amongst these three ethnic groups. This information will be helpful in designing a protocol for genetic testing for deafness and for achieving accurate molecular diagnoses in northwest China.
本研究旨在探讨中国西北地区回族、藏族和土族听力障碍人群中非综合征性听力障碍(HI)的分子病因。本研究共纳入 283 名来自中国西北地区特殊教育学校的非综合征性听力障碍患者,均为无亲缘关系个体。采用 SNPscan 技术检测 3 个常见耳聋相关基因(间隙连接蛋白β2(GJB2)、溶质载体家族 26 成员 4(SLC26A4)和线粒体编码 12S RNA(mtDNA12SrRNA))中的单核苷酸多态性(SNP)。在回族患者中,GJB2 突变的检出率为 14.89%,藏族患者为 9.37%,土族患者为 11.83%。在回族和土族患者中,最常见的 GJB2 突变是 c.235delC。在藏族患者中,c.109G>A SNP 的等位基因频率最高。在回族患者中,SLC26A4 突变的检出率为 10.64%,藏族患者为 6.25%,土族患者为 8.6%。最常见的 SLC26A4 突变是 c.919-2A>G,在回族、藏族和土族患者中,其次最常见的 SLC26A4 突变分别是 c.1517T>G、c.1226G>A 和 c.2168A>G。在回族、藏族和土族患者中,mtDNA12SrRNA 的突变率分别为 1.06%、5.21%和 5.38%。这些发现表明,这些耳聋相关基因的突变谱在这三个民族中是独特的。这些信息将有助于设计在中国西北地区进行耳聋基因检测的方案,并实现准确的分子诊断。