Gurramkonda Venkatesh Babu, Syed Altaf Hussain, Murthy Jyotsna, Lakkakula Bhaskar V K S
Sri Ramachandra University, Department of Biomedical Sciences, Chennai, India.
Sri Ramachandra University, Department of Plastic Surgery, Chennai, India.
Braz J Otorhinolaryngol. 2018 Jul-Aug;84(4):473-477. doi: 10.1016/j.bjorl.2017.05.011. Epub 2017 Jun 26.
Transcription factors are very diverse family of proteins involved in activating or repressing the transcription of a gene at a given time. Several studies using animal models demonstrated the role of transcription factor genes in craniofacial development.
We aimed to investigate the association of IRF6 intron-6 polymorphism in the non-syndromic cleft lip with or without palate in a South Indian population.
173 unrelated nonsyndromic cleft lip with or without cleft palate patients and 176 controls without clefts patients were genotyped for IRF6 rs2235375 variant by allele-specific amplification using the KASPar single nucleotide polymorphism genotyping system. The association between interferon regulatory factor-6 gene intron-6 dbSNP208032210:g.G>C (rs2235375) single nucleotide polymorphism and non-syndromic cleft lip with or without palate risk was investigated by chi-square test.
There were significant differences in genotype or allele frequencies of rs2235375 single nucleotide polymorphism between controls and cases with non-syndromic cleft lip with or without palate. IRF6 rs2235375 variant was significantly associated with increased risk of non-syndromic cleft lip with or without palate in co-dominant, dominant (OR: 1.19; 95% CI 1.03-2.51; p=0.034) and allelic models (OR: 1.40; 95% CI 1.04-1.90; p=0.028). When subset analysis was applied significantly increased risk was observed in cleft palate only group (OR dominant: 4.33; 95% CI 1.44-12.97; p=0.005).
These results suggest that IRF6 rs2235375 SNP play a major role in the pathogenesis and risk of developing non-syndromic cleft lip with or without palate.
转录因子是一类非常多样的蛋白质家族,参与在特定时间激活或抑制基因的转录。多项使用动物模型的研究证明了转录因子基因在颅面发育中的作用。
我们旨在研究南印度人群中,IRF6基因内含子6多态性与非综合征性唇腭裂(伴或不伴腭裂)之间的关联。
使用KASPar单核苷酸多态性基因分型系统,通过等位基因特异性扩增对173例无亲缘关系的非综合征性唇腭裂患者和176例无腭裂的对照患者进行IRF6 rs2235375变异的基因分型。采用卡方检验研究干扰素调节因子6基因内含子6 dbSNP208032210:g.G>C(rs2235375)单核苷酸多态性与非综合征性唇腭裂(伴或不伴腭裂)风险之间的关联。
rs2235375单核苷酸多态性的基因型或等位基因频率在对照组与非综合征性唇腭裂(伴或不伴腭裂)病例组之间存在显著差异。IRF6 rs2235375变异在共显性、显性(比值比:1.19;95%置信区间1.03 - 2.51;p = 0.034)和等位基因模型(比值比:1.40;95%置信区间1.04 - 1.90;p = 0.028)中与非综合征性唇腭裂(伴或不伴腭裂)风险增加显著相关。当进行亚组分析时,仅腭裂组观察到风险显著增加(显性比值比:4.33;95%置信区间1.44 - 12.97;p = 0.005)。
这些结果表明,IRF6 rs2235375单核苷酸多态性在非综合征性唇腭裂(伴或不伴腭裂)的发病机制和发病风险中起主要作用。