CSIR-Centre for Cellular and Molecular Biology, Hyderabad, India.
DNA Cell Biol. 2012 Apr;31(4):616-24. doi: 10.1089/dna.2011.1366. Epub 2011 Oct 21.
We sought to determine the frequency of the genetic variations in the Troponin T (TNNT2) gene and its association in Indian cardiomyopathy patients. Sequencing of the entire TNNT2 gene in 162 hypertrophic cardiomyopathy (HCM) patients, along with 179 healthy controls, revealed a total of 15 variants. These included an A28V missense mutation, a novel single-nucleotide polymorphism (SNP) (g.7239;G→A) predicted to disturb the splicing significantly, three SNPs, rs3729547 (C→T), rs3729843 (G→A), rs3729842 (C→T), which were in high linkage disequilibrium, and a 5 bp polymorphism that skipped exon 4 during splicing, which was found to be significantly higher in HCM patients (del/del genotype, p=0.00011; deletion allele, p=0.00008). Further studies on the 5 bp polymorphism in 2092 randomly selected individuals belonging to 39 ethnic and endogamous populations from 19 states of India, and representing the major linguistic Indian families, revealed that the South and the Northwest Indians have a high frequency of 5 bp deletions. The missense mutations in TNNT2 are responsible for 15%-20% of familial HCM by impairing the function of the heart muscle. However, other than the 5 bp polymorphism, our comprehensive study on the Indian HCM patients have lowered the occurrence and overall prevalence of supposedly more aggressive and worst disease causing percentage of missense mutations in TNNT2 dramatically.
我们旨在确定肌钙蛋白 T(TNNT2)基因的遗传变异的频率及其在印度心肌病患者中的相关性。对 162 例肥厚型心肌病(HCM)患者和 179 例健康对照者的整个 TNNT2 基因进行测序,共发现 15 种变异。其中包括一个 A28V 错义突变,一个新的单核苷酸多态性(SNP)(g.7239; G→A),预计会显著干扰剪接,三个 SNP,rs3729547(C→T),rs3729843(G→A),rs3729842(C→T),它们高度连锁不平衡,还有一个 5bp 外显子跳跃多态性,在剪接过程中跳过外显子 4,在 HCM 患者中发现明显更高(del/del 基因型,p=0.00011;缺失等位基因,p=0.00008)。进一步对来自印度 19 个州的 39 个种族和内婚群体的 2092 名随机个体进行了 5bp 多态性研究,代表了主要的印度语言家族,结果显示,南部和西北部的印度人有很高的 5bp 缺失频率。TNNT2 的错义突变通过损害心肌的功能,导致 15%-20%的家族性 HCM。然而,除了 5bp 多态性外,我们对印度 HCM 患者的综合研究大大降低了 TNNT2 中假定更具攻击性和导致病情恶化的错义突变的发生率和总体患病率。